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Showing metabocard for Isoleucine (HMDB0000172)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-07-07 20:53:57 UTC
HMDB IDHMDB0000172
Secondary Accession Numbers
  • HMDB00172
Metabolite Identification
Common NameIsoleucine
DescriptionIsoleucine (Ile) or L-isoleucine is an alpha-amino acid. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon). Amino acids are organic compounds that contain amino (-NH2) and carboxyl (-COOH) functional groups, along with a side chain (R group) specific to each amino acid. L-isolecuine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Isoleucine is found in all organisms ranging from bacteria to plants to animals. It is classified as a non-polar, uncharged (at physiological pH) aliphatic amino acid. Isoleucine is an essential amino acid in humans, meaning the body cannot synthesize it and that it must be obtained from the diet. In plants and microorganisms, isoleucine is synthesized starting from pyruvate and alpha-ketobutyrate. Isoleucine is classified as a branched chain amino acid (BCAA). BCAAs include three amino acids: isoleucine, leucine and valine. They are alpha amino acids whose carbon structure is marked by a beta branch point. Despite their structural similarities, BCAAs have different metabolic routes, with valine going solely to carbohydrates (glucogenic), leucine solely to fats (ketogenic) and isoleucine being both a glucogenic and a ketogenic amino acid. Isoleucine is catabolized via with alpha-ketoglutarate where upon it is oxidized and split into propionyl-CoA and acetyl-CoA. Propionyl-CoA is converted into succinyl-CoA, a TCA cycle intermediate which can be converted into oxaloacetate for gluconeogenesis (hence glucogenic). The acetyl-CoA can be fed into the TCA cycle by condensing with oxaloacetate to form citrate or used in the synthesis of ketone bodies or fatty acids. The different metabolism of BCAAs accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine are required respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. BCAAs are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia. An inability to break down isoleucine, along with other amino acids, is associated with maple syrup urine disease (MSUD) (PMID: 34125801 ). Isoleucine, like other BCAAs, is associated with insulin resistance. In particular, higher levels of isoleucine are observed in the blood of diabetic mice, rats, and humans (PMID 25287287 ). Mice fed an isoleucine deprivation diet for one day have improved insulin sensitivity, and feeding of an isoleucine deprivation diet for one week significantly decreases blood glucose levels (PMID: 24684822 ).
Structure
Data?1676999675
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000172 (Isoleucine)


  Mrv1652309272007352D          

  9  8  0  0  1  0            999 V2000
    5.2224   -0.0784    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    1.1592    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.7935   -0.9034    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    0.3341    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
    3.7935   -0.0784    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.3645   -0.0784    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    1.1592    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6500    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  9  1  0  0  0  0
  2  9  2  0  0  0  0
  5  3  1  1  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
  4  7  1  6  0  0  0
  5  9  1  0  0  0  0
  6  8  1  0  0  0  0
M  END
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3D MOL for HMDB0000172 (Isoleucine)

HMDB0000172
     RDKit          3D
L-Isoleucine
 22 21  0  0  0  0  0  0  0  0999 V2000
   -1.9483    1.3874   -0.2015 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7094   -0.0928   -0.0954 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2927   -0.4903   -0.3933 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.2345   -2.0164   -0.2386 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7018    0.0749    0.5808 C   0  0  2  0  0  0  0  0  0  0  0  0
    0.7185    1.5085    0.6256 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.0588   -0.3788    0.1206 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3447   -1.5974   -0.0157 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.0704    0.5308   -0.1787 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9257    1.5414   -0.7238 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0637    1.7905    0.8190 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1964    1.9172   -0.8110 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9488   -0.4780    0.9220 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3631   -0.6450   -0.8022 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0263   -0.2743   -1.4269 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4097   -2.4817   -0.9859 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2615   -2.4565   -0.3917 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0471   -2.2815    0.8132 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5202   -0.3244    1.5957 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3611    1.8613   -0.1223 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.2068    1.7723    1.5347 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4789    1.1329    0.5295 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  1  0
  5  7  1  0
  7  8  2  0
  7  9  1  0
  1 10  1  0
  1 11  1  0
  1 12  1  0
  2 13  1  0
  2 14  1  0
  3 15  1  6
  4 16  1  0
  4 17  1  0
  4 18  1  0
  5 19  1  1
  6 20  1  0
  6 21  1  0
  9 22  1  0
M  END
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3D SDF for HMDB0000172 (Isoleucine)


  Mrv1652309272007352D          

  9  8  0  0  1  0            999 V2000
    5.2224   -0.0784    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    1.1592    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.7935   -0.9034    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    0.3341    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
    3.7935   -0.0784    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.3645   -0.0784    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    1.1592    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6500    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  9  1  0  0  0  0
  2  9  2  0  0  0  0
  5  3  1  1  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
  4  7  1  6  0  0  0
  5  9  1  0  0  0  0
  6  8  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000172

> <DATABASE_NAME>
hmdb

> <SMILES>
CC[C@H](C)[C@H](N)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1

> <INCHI_KEY>
AGPKZVBTJJNPAG-WHFBIAKZSA-N

> <FORMULA>
C6H13NO2

> <MOLECULAR_WEIGHT>
131.1729

> <EXACT_MASS>
131.094628665

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
22

> <JCHEM_AVERAGE_POLARIZABILITY>
14.11454343235902

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2S,3S)-2-amino-3-methylpentanoic acid

> <ALOGPS_LOGP>
-1.73

> <JCHEM_LOGP>
-1.5084066056000942

> <ALOGPS_LOGS>
-0.06

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
2.7913082723326537

> <JCHEM_PKA_STRONGEST_BASIC>
9.591345628135606

> <JCHEM_POLAR_SURFACE_AREA>
63.31999999999999

> <JCHEM_REFRACTIVITY>
34.093900000000005

> <JCHEM_ROTATABLE_BOND_COUNT>
3

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.14e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
L-isoleucine

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0000172 (Isoleucine)

HMDB0000172
     RDKit          3D
L-Isoleucine
 22 21  0  0  0  0  0  0  0  0999 V2000
   -1.9483    1.3874   -0.2015 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7094   -0.0928   -0.0954 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2927   -0.4903   -0.3933 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.2345   -2.0164   -0.2386 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7018    0.0749    0.5808 C   0  0  2  0  0  0  0  0  0  0  0  0
    0.7185    1.5085    0.6256 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.0588   -0.3788    0.1206 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3447   -1.5974   -0.0157 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.0704    0.5308   -0.1787 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9257    1.5414   -0.7238 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0637    1.7905    0.8190 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1964    1.9172   -0.8110 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9488   -0.4780    0.9220 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3631   -0.6450   -0.8022 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0263   -0.2743   -1.4269 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4097   -2.4817   -0.9859 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2615   -2.4565   -0.3917 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0471   -2.2815    0.8132 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5202   -0.3244    1.5957 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3611    1.8613   -0.1223 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.2068    1.7723    1.5347 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4789    1.1329    0.5295 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  1  0
  5  7  1  0
  7  8  2  0
  7  9  1  0
  1 10  1  0
  1 11  1  0
  1 12  1  0
  2 13  1  0
  2 14  1  0
  3 15  1  6
  4 16  1  0
  4 17  1  0
  4 18  1  0
  5 19  1  1
  6 20  1  0
  6 21  1  0
  9 22  1  0
M  END
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PDB for HMDB0000172 (Isoleucine)

HEADER    PROTEIN                                 27-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-SEP-20         0                                  
HETATM    1  O   UNK     0       9.748  -0.146   0.000  0.00  0.00           O+0
HETATM    2  O   UNK     0       8.415   2.164   0.000  0.00  0.00           O+0
HETATM    3  N   UNK     0       7.081  -1.686   0.000  0.00  0.00           N+0
HETATM    4  C   UNK     0       5.748   0.624   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0       7.081  -0.146   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0       4.414  -0.146   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0       5.748   2.164   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0       3.080   0.624   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0       8.415   0.624   0.000  0.00  0.00           C+0
CONECT    1    9                                                            
CONECT    2    9                                                            
CONECT    3    5                                                            
CONECT    4    5    6    7                                                  
CONECT    5    3    4    9                                                  
CONECT    6    4    8                                                       
CONECT    7    4                                                            
CONECT    8    6                                                            
CONECT    9    1    2    5                                                  
MASTER        0    0    0    0    0    0    0    0    9    0   16    0
END
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3D PDB for HMDB0000172 (Isoleucine)

COMPND    HMDB0000172
HETATM    1  C1  UNL     1      -1.948   1.387  -0.202  1.00  0.00           C  
HETATM    2  C2  UNL     1      -1.709  -0.093  -0.095  1.00  0.00           C  
HETATM    3  C3  UNL     1      -0.293  -0.490  -0.393  1.00  0.00           C  
HETATM    4  C4  UNL     1      -0.235  -2.016  -0.239  1.00  0.00           C  
HETATM    5  C5  UNL     1       0.702   0.075   0.581  1.00  0.00           C  
HETATM    6  N1  UNL     1       0.719   1.509   0.626  1.00  0.00           N  
HETATM    7  C6  UNL     1       2.059  -0.379   0.121  1.00  0.00           C  
HETATM    8  O1  UNL     1       2.345  -1.597  -0.016  1.00  0.00           O  
HETATM    9  O2  UNL     1       3.070   0.531  -0.179  1.00  0.00           O  
HETATM   10  H1  UNL     1      -2.926   1.541  -0.724  1.00  0.00           H  
HETATM   11  H2  UNL     1      -2.064   1.790   0.819  1.00  0.00           H  
HETATM   12  H3  UNL     1      -1.196   1.917  -0.811  1.00  0.00           H  
HETATM   13  H4  UNL     1      -1.949  -0.478   0.922  1.00  0.00           H  
HETATM   14  H5  UNL     1      -2.363  -0.645  -0.802  1.00  0.00           H  
HETATM   15  H6  UNL     1       0.026  -0.274  -1.427  1.00  0.00           H  
HETATM   16  H7  UNL     1       0.410  -2.482  -0.986  1.00  0.00           H  
HETATM   17  H8  UNL     1      -1.262  -2.457  -0.392  1.00  0.00           H  
HETATM   18  H9  UNL     1       0.047  -2.281   0.813  1.00  0.00           H  
HETATM   19  H10 UNL     1       0.520  -0.324   1.596  1.00  0.00           H  
HETATM   20  H11 UNL     1       1.361   1.861  -0.122  1.00  0.00           H  
HETATM   21  H12 UNL     1       1.207   1.772   1.535  1.00  0.00           H  
HETATM   22  H13 UNL     1       3.479   1.133   0.530  1.00  0.00           H  
CONECT    1    2   10   11   12
CONECT    2    3   13   14
CONECT    3    4    5   15
CONECT    4   16   17   18
CONECT    5    6    7   19
CONECT    6   20   21
CONECT    7    8    8    9
CONECT    9   22
END
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SMILES for HMDB0000172 (Isoleucine)

CC[C@H](C)[C@H](N)C(O)=O
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INCHI for HMDB0000172 (Isoleucine)

InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
ValueSource
(2S,3S)-2-Amino-3-methylpentanoic acidChEBI
2-Amino-3-methylvaleric acidChEBI
alpha-Amino-beta-methylvaleric acidChEBI
IChEBI
IleChEBI
ISOLEUCINEChEBI
(2S,3S)-2-Amino-3-methylpentanoateGenerator
2-Amino-3-methylvalerateGenerator
a-Amino-b-methylvalerateGenerator
a-Amino-b-methylvaleric acidGenerator
alpha-Amino-beta-methylvalerateGenerator
Α-amino-β-methylvalerateGenerator
Α-amino-β-methylvaleric acidGenerator
(2S,3S)-2-Amino-3-methyl-pentanoateHMDB
(2S,3S)-2-Amino-3-methyl-pentanoic acidHMDB
(2S,3S)-a-Amino-b-methyl-N-valerateHMDB
(2S,3S)-a-Amino-b-methyl-N-valeric acidHMDB
(2S,3S)-a-Amino-b-methylvalerateHMDB
(2S,3S)-a-Amino-b-methylvaleric acidHMDB
(2S,3S)-Alph-amino-beta-methylvalerateHMDB
(2S,3S)-Alph-amino-beta-methylvaleric acidHMDB
(2S,3S)-alpha-Amino-beta-merthyl-N-valerateHMDB
(2S,3S)-alpha-Amino-beta-merthyl-N-valeric acidHMDB
(2S,3S)-alpha-Amino-beta-merthylvalerateHMDB
(2S,3S)-alpha-Amino-beta-merthylvaleric acidHMDB
(2S,3S)-alpha-Amino-beta-methyl-N-valerateHMDB
(2S,3S)-alpha-Amino-beta-methyl-N-valeric acidHMDB
(2S,3S)-alpha-Amino-beta-methylvalerateHMDB
(2S,3S)-alpha-Amino-beta-methylvaleric acidHMDB
(S)-IsoleucineHMDB
(S,S)-IsoleucineHMDB
2-Amino-3-methylpentanoateHMDB
2-Amino-3-methylpentanoic acidHMDB
2S,3S-IsoleucineHMDB
Erythro-L-isoleucineHMDB
Iso-leucineHMDB
L-(+)-IsoleucineHMDB
L-IleHMDB
[S-(R*,r*)]-2-amino-3-methylpentanoateHMDB
[S-(R*,r*)]-2-amino-3-methylpentanoic acidHMDB
Isoleucine, L-isomerHMDB
AlloisoleucineHMDB
Isoleucine, L isomerHMDB
L-Isomer isoleucineHMDB
2S-Amino-3S-methylpentanoateHMDB
L-IsoleucineKEGG
Chemical FormulaC6H13NO2
Average Molecular Weight131.1729
Monoisotopic Molecular Weight131.094628665
IUPAC Name(2S,3S)-2-amino-3-methylpentanoic acid
Traditional NameL-isoleucine
CAS Registry Number73-32-5
SMILES
CC[C@H](C)[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1
InChI KeyAGPKZVBTJJNPAG-WHFBIAKZSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as isoleucine and derivatives. Isoleucine and derivatives are compounds containing isoleucine or a derivative thereof resulting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentIsoleucine and derivatives
Alternative Parents
Substituents
  • Isoleucine or derivatives
  • Alpha-amino acid
  • L-alpha-amino acid
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxide
  • Organopnictogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Organoleptic effect
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Biological roleIndustrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point285.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility35 mg/mLHuman Metabolome Project
LogP-1.70HANSCH,C ET AL. (1995)
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Astarita_neg129.030932474
[M-H]-Baker132.6230932474
[M-H]-McLean129.930932474
[M-H]-MetCCS_test_neg126.730932474
[M+H]+Baker134.830932474
[M+H]+McLean133.030932474
[M+H]+Astarita_pos128.730932474
[M-H]-Not Available130.8http://allccs.zhulab.cn/database/detail?ID=AllCCS00000078
[M+H]+Not Available136.967http://allccs.zhulab.cn/database/detail?ID=AllCCS00000078
Predicted Molecular Properties
PropertyValueSource
Water Solubility114 g/LALOGPS
logP-1.7ALOGPS
logP-1.5ChemAxon
logS-0.06ALOGPS
pKa (Strongest Acidic)2.79ChemAxon
pKa (Strongest Basic)9.59ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.09 m³·mol⁻¹ChemAxon
Polarizability14.11 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+130.72331661259
DarkChem[M-H]-127.12331661259
AllCCS[M+H]+132.34232859911
AllCCS[M-H]-129.05832859911
DeepCCS[M+H]+131.21730932474
DeepCCS[M-H]-127.3930932474
DeepCCS[M-2H]-164.84830932474
DeepCCS[M+Na]+140.38630932474
AllCCS[M+H]+132.332859911
AllCCS[M+H-H2O]+128.232859911
AllCCS[M+NH4]+136.232859911
AllCCS[M+Na]+137.332859911
AllCCS[M-H]-129.132859911
AllCCS[M+Na-2H]-131.932859911
AllCCS[M+HCOO]-135.032859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
L-IsoleucineCC[C@H](C)[C@H](N)C(O)=O1985.0Standard polar33892256
L-IsoleucineCC[C@H](C)[C@H](N)C(O)=O1164.3Standard non polar33892256
L-IsoleucineCC[C@H](C)[C@H](N)C(O)=O1292.7Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
L-Isoleucine,1TMS,isomer #1CC[C@H](C)[C@H](N)C(=O)O[Si](C)(C)C1180.4Semi standard non polar33892256
L-Isoleucine,1TMS,isomer #2CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O1292.1Semi standard non polar33892256
L-Isoleucine,2TMS,isomer #1CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1316.6Semi standard non polar33892256
L-Isoleucine,2TMS,isomer #1CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1308.4Standard non polar33892256
L-Isoleucine,2TMS,isomer #1CC[C@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1419.5Standard polar33892256
L-Isoleucine,2TMS,isomer #2CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1480.8Semi standard non polar33892256
L-Isoleucine,2TMS,isomer #2CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1322.9Standard non polar33892256
L-Isoleucine,2TMS,isomer #2CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1511.7Standard polar33892256
L-Isoleucine,3TMS,isomer #1CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1501.3Semi standard non polar33892256
L-Isoleucine,3TMS,isomer #1CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1434.9Standard non polar33892256
L-Isoleucine,3TMS,isomer #1CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1401.2Standard polar33892256
L-Isoleucine,1TBDMS,isomer #1CC[C@H](C)[C@H](N)C(=O)O[Si](C)(C)C(C)(C)C1396.9Semi standard non polar33892256
L-Isoleucine,1TBDMS,isomer #2CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O1519.3Semi standard non polar33892256
L-Isoleucine,2TBDMS,isomer #1CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1749.9Semi standard non polar33892256
L-Isoleucine,2TBDMS,isomer #1CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1724.7Standard non polar33892256
L-Isoleucine,2TBDMS,isomer #1CC[C@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1722.9Standard polar33892256
L-Isoleucine,2TBDMS,isomer #2CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1878.2Semi standard non polar33892256
L-Isoleucine,2TBDMS,isomer #2CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1758.8Standard non polar33892256
L-Isoleucine,2TBDMS,isomer #2CC[C@H](C)[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1750.4Standard polar33892256
L-Isoleucine,3TBDMS,isomer #1CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2161.4Semi standard non polar33892256
L-Isoleucine,3TBDMS,isomer #1CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2055.1Standard non polar33892256
L-Isoleucine,3TBDMS,isomer #1CC[C@H](C)[C@@H](C(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1828.2Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0930000000-e78f845bb2a8d47364762014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0910000000-de9162d149073d0e2a372014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0920000000-599e61f8ccdb6525c7a92014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0a4i-0910000000-742e44c426c0d6c2a9ac2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-05fr-8910000000-dbb33e0f02ac2ca5fedd2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine EI-B (Non-derivatized)splash10-0a4i-0920000000-37690f426455ac41e2c22017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)splash10-0a4i-0930000000-e78f845bb2a8d47364762017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)splash10-0a4i-0910000000-de9162d149073d0e2a372017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)splash10-0a4i-0920000000-599e61f8ccdb6525c7a92017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)splash10-0a4i-0910000000-742e44c426c0d6c2a9ac2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-QQ (Non-derivatized)splash10-0udi-2392000000-74cca1eb265b8e3d7f432017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)splash10-05fr-8910000000-dbb33e0f02ac2ca5fedd2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)splash10-000i-9100000000-27891dff695c5db537962017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Isoleucine GC-EI-TOF (Non-derivatized)splash10-0a4i-0910000000-43ec1890f6dbd6aea6572017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isoleucine GC-MS (Non-derivatized) - 70eV, Positivesplash10-00b9-9100000000-40bc9ef43da5f18be8832016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isoleucine GC-MS (1 TMS) - 70eV, Positivesplash10-000i-9200000000-ae8694c0f3e4dcc608f92017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isoleucine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isoleucine GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isoleucine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isoleucine GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
MSMass Spectrum (Electron Ionization)splash10-004r-9000000000-34d4d4cb7042da231eb42014-09-20Not AvailableView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-000i-9300000000-8a23b3e62231eb65f80f2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-00ko-9000000000-ac7e71578d7e1d0e8ce92012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-052f-9000000000-2586d4a089921dd977ed2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-001i-0900000000-5b11521ff6a631376d2b2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-000i-9000000000-3c352f229e4067fcd4892012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-000i-9000000000-8a7ef48fc0c1b6f845c22012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-0002-0920000000-2e11aa1c7a5defc386db2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-001i-0900000000-b63ac9cf06eda4c54a812012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-000i-9000000000-e5b0c8c6b09f541d6dbf2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-000i-9000000000-d89d16d5c2242e44ec632012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-000i-9000000000-5de583142c7bdb381c8f2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOFsplash10-001i-0900000000-8c75bde35c4a4073ee652012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOFsplash10-001i-0900000000-233a9862f616afea2d172012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOFsplash10-001i-1900000000-375f35065b82b13e6d6e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOFsplash10-00dl-9000000000-d0d4a9f90fe2aca744832012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOFsplash10-0006-9000000000-0018f47571feaf232ff82012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive-QTOFsplash10-001r-7900000000-278dc67396114be331ba2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive-QTOFsplash10-000i-9000000000-e26c042aa6231eeca0712012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive-QTOFsplash10-014r-9000000000-b6c1752fd3fbccb3d1c52012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive-QTOFsplash10-05mo-9000000000-0569c3162621252ed0a82012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive-QTOFsplash10-052f-9000000000-3d52b5d56d3fab45276e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isoleucine CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positive-QTOFsplash10-001i-0900000000-720554d58264a9cfdb672012-08-31HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isoleucine 10V, Positive-QTOFsplash10-0019-9500000000-00268b2694cde281641b2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isoleucine 20V, Positive-QTOFsplash10-000i-9000000000-4014793e17e0290790a42016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isoleucine 40V, Positive-QTOFsplash10-0a4i-9000000000-eb9a97a14461e524e76e2016-09-12Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Experimental 1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, experimental)2021-10-10Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Quadrupole Ion Trap, ESI+, Adduct: [M+H]+)2022-02-11FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • All Tissues
  • Placenta
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified40.0 +/- 20.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified69.0 +/- 13.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified64.0 +/- 13.0 uMChildren (1-13 years old)FemaleNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified84.0 +/- 18.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified44.0 +/- 6.0 uMChildren (1-13 years old)Not SpecifiedNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified81.0 +/- 18.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified56.0 +/- 12.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified28-84 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified20-90 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
 details
BloodDetected and Quantified25-87 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
 details
BloodDetected and Quantified25-100 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
 details
BloodDetected and Quantified35-100 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
 details
BloodDetected and Quantified37-39 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified83.1 (72.1-96.5) uMNewborn (0-30 days old)Not Available
Normal		 details
BloodDetected and Quantified38-94 uMAdolescent (13-18 years old)Not Specified
Normal		 details
BloodDetected and Quantified22-107 uMChildren (1-13 years old)Not Specified
Normal		 details
BloodDetected and Quantified68 +/- 11 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified22-107 uMChildren (1 - 18 years old)Not SpecifiedNormal details
BloodDetected and Quantified30-108 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified53 +/- 7 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified31-83 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified92.1 (71.4-116) uMInfant (0-1 year old)Not Available
Normal		 details
BloodDetected and Quantified39 +/- 8 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified76.5 +/- 18.0 uMChildren (1 - 13 years old)Not Specified
Normal		 details
BloodDetected and Quantified64 +/- 13 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified40.00-100.0 uMAdult (>18 years old)Both
Normal		 details
BloodDetected and Quantified77.5 +/- 15.4 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified84 +/- 18 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified51.1(44.6-60.2) uMChildren (1-13 uears old)Both
Normal		 details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
BloodDetected and Quantified62.0 (48.0-76.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified60.7 +/- 18.6 uMAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified7.9 +/- 3.3 uMAdult (>18 years old)Female
Normal		 details
Cerebrospinal Fluid (CSF)Detected and Quantified4.9 +/- 0.6 uMNewborn (0-30 days old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified7.0 +/- 5.0 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3.9 +/- 1.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 details
Cerebrospinal Fluid (CSF)Detected and Quantified5.5 +/- 0.64 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8.4 +/- 2.6 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8.4 +/- 2.5 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.3 +/- 3.4 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1.2-7.5 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
 details
Cerebrospinal Fluid (CSF)Detected and Quantified0.3-9.3 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
 details
Cerebrospinal Fluid (CSF)Detected and Quantified5.77 (3.30-8.24) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified13.5 (8.8-18.2) uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected and Quantified4171 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected and Quantified59209.384 (23912.460-94506.309) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal		 details
FecesDetected and Quantified41395.746 (17686.580-65104.911) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal		 details
FecesDetected and Quantified60810.325 (32222.103-89398.547) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedBoth
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
FecesDetected and Quantified24.853 +/- 27.140 nmol/g wet fecesNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected and Quantified1090 +/- 650 nmol/g wet fecesAdult (>18 years old)Both
Normal		 details
FecesDetected and Quantified1470 +/- 620 nmol/g wet fecesAdult (>18 years old)Female
Normal		 details
FecesDetected and Quantified160 +/- 160 nmol/g wet fecesAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal		 details
FecesDetected and Quantified3733 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected and Quantified3644 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal		 details
SalivaDetected and Quantified6.60 +/- 9.47 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected and Quantified0.0890 +/- 0.330 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected and Quantified5.4 +/- 5.0 uMAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified9.0 +/- 3.5 uMAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified2.3 +/- 3.4 uMAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified25.53 +/- 17.89 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
 details
SalivaDetected and Quantified6.0 +/- 5.2 uMAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified2.36 +/- 2.74 uMAdult (>18 years old)FemaleNormal details
SweatDetected and Quantified40-260 uMAdult (60 years old)Male
Normal		 details
SweatDetected and Quantified40 uMAdult (40 years old)Male
Normal		 details
SweatDetected but not QuantifiedNot QuantifiedAdult BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleNormal details
UrineDetected and Quantified3.93 +/- 3.136 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
 details
UrineDetected and Quantified<3.85 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified1.64 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<3.17 umol/mmol creatinineAdolescent (13-18 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.78-1.4 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified3.05-16.78 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
 details
UrineDetected and Quantified1.579 (0.789-2.368) umol/mmol creatinineAdult (>18 years old)BothNormal
      Not Available
 details
UrineDetected and Quantified<2.49 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.43-14.07 umol/mmol creatinineNewborn (0-30 days old)Male
Normal		 details
UrineDetected and Quantified0.86-16.19 umol/mmol creatinineNewborn (0-30 days old)Female
Normal		 details
UrineDetected and Quantified4.82 +/- 4.26 umol/mmol creatinineNewborn (0-30 days old)Male
Normal		 details
UrineDetected and Quantified5.68 +/- 4.59 umol/mmol creatinineNewborn (0-30 days old)Female
Normal		 details
UrineDetected and Quantified6.62 +/- 4.81 umol/mmol creatinineChildren (0-2 years old)Female
Normal		 details
UrineDetected and Quantified6.83 +/- 4.78 umol/mmol creatinineChildren (0-2 years old)Male
Normal		 details
UrineDetected and Quantified1.3 (0.4-2.6) umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
UrineDetected and Quantified0.0086 - 0.0339 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.41 umol/mmol creatinineAdult (>18 years old)Male
Normal
    • Shaykhutdinov RA,...
 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
UrineDetected and Quantified3.75 +/- 2.75 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified<9.73 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified< 1.0 umol/mmol creatinineNewborn (0-30 days old)Both
Normal		 details
UrineDetected and Quantified<1.0 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal		 details
UrineDetected and Quantified<1.0 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal		 details
UrineDetected and Quantified0.94 +/- 0.67 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
 details
UrineDetected and Quantified1.8 +/- 1.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
 details
UrineDetected and Quantified3.0 +/- 1.5 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
 details
UrineDetected and Quantified3.55 +/- 1.85 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.3 (0.5-2.7) umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
UrineDetected and Quantified2.35 (1.32-4.29) umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
 details
UrineDetected and Quantified<8.82 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.71-1.1 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
UrineDetected and Quantified0.9 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified3.7308 +/- 1.4276 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
 details
UrineDetected and Quantified35.39 +/- 33.92 umol/mmol creatinineNewborn (0-30 days old)Both
Normal		 details
UrineDetected and Quantified46.81 +/- 29.40 umol/mmol creatinineNewborn (0-30 days old)Both
Normal		 details
UrineDetected and Quantified48.16 +/- 29.40 umol/mmol creatinineNewborn (0-30 days old)Both
Normal		 details
UrineDetected and Quantified<7.013 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified0 - 6.11 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0 - 5.09 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0 - 6.11 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0 - 6.11 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0 - 16.62 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0 - 6.11 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0 - 4.07 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified0.29-7.30 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0 - 4.07 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified2.73 +/- 1.52 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified2.93 +/- 1.42 umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Oesophageal cancer		 details
BloodDetected and Quantified44.6 +/- 21.5 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified477 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
 details
BloodDetected and Quantified103 uMChildren (1-13 years old)FemaleHyperlysinemia II or Saccharopinuria details
BloodDetected and Quantified41.1 +/- 10.7 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18		 details
BloodDetected and Quantified38.6 +/- 16.6 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified37.6 (9.6) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified46.2 (19.3) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified39.5 (7-51) uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
BloodDetected and Quantified282.0705 uMInfant (0-1 year old)MaleDihydrolipoamide Dehydrogenase Deficiency details
BloodDetected and Quantified10-23 uMAdolescent (13-18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified10-26 uMAdult (>18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified77 +/- 14 uMAdult (>18 years old)MaleSchizophrenia details
BloodDetected and Quantified9-57 uMChildren (1-13 years old)BothBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified64 +/- 15 uMAdult (>18 years old)FemaleSchizophrenia details
BloodDetected and Quantified43.24 (16.72) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified40.9 (11.7) uMAdult (>18 years old)FemalePregnancy details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
BloodDetected and Quantified492 uMNewborn (0-30 days old)FemaleMaple syrup urine disease details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Pancreatic cancer		 details
BloodDetected and Quantified237-794 uMNewborn (0-30 days old)BothMaple syrup urine disease details
BloodDetected and Quantified99.0 +/- 65.3 uMChildren (1 - 13 years old)Not Specified
Maple syrup urine disease		 details
BloodDetected and Quantified58.6(49.2-70.0) uMChildren (1-13 uears old)Both
Environmental enteric dysfunction		 details
BloodDetected and Quantified1495.2 +/- 373.0 uMElderly (>65 years old)BothAlzheimer's disease details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Pancreatic cancer		 details
BloodDetected and Quantified40.3 (38.0-42.6) uMAdult (>18 years old)Both
Epilepsy		 details
BloodDetected and Quantified42.7 (38.1-47.3) uMAdult (>18 years old)Both
Epilepsy		 details
BloodDetected and Quantified6100.00 (200.00-12000.00) uMChildren (1-13 years old)BothMaple syrup urine disease (MSUD)
    • MetaGene: Metabol...
 details
BloodDetected and Quantified166.0 +/- 5.0 uMAdult (>18 years old)Both
Heart failure		 details
Cerebrospinal Fluid (CSF)Detected and Quantified216 uMNewborn (0-30 days old)FemaleMaple syrup urine disease details
Cerebrospinal Fluid (CSF)Detected and Quantified338 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
 details
Cerebrospinal Fluid (CSF)Detected and Quantified6.0 +/- 0.65 uMAdult (>18 years old)BothSchizophrenia details
Cerebrospinal Fluid (CSF)Detected and Quantified2.4 +/- 0.65 uMAdult (>18 years old)BothAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified7.1 +/- 4.2 uMChildren (1-13 years old)Not Specified
Leukemia		 details
Cerebrospinal Fluid (CSF)Detected and Quantified5.9 +/- 4.0 uMChildren (1-13 years old)Not SpecifiedLeukemia details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothAutism details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Irritable bowel syndrome		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative colitis		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Cryptosporidium infection		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUnclassified IBD details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothIrritable Bowel Syndrome details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Crohn’s Disease		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative Colitis		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothCrohn's disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothUlcerative colitis details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleAttachment loss  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleMissing teeth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MalePeriodontal Probing Depth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleBreast cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedPancreatic cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedPeriodontal diseases details
SalivaDetected and Quantified0.83 +/- 0.21 uMAdult (>18 years old)MaleAlzheimer's disease details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral squamous cell carcinoma (OSCC) details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral leukoplakia (OLK) details
SalivaDetected and Quantified1.22 +/- 0.30 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
SalivaDetected and Quantified1.54 +/- 0.98 uMAdult (>18 years old)BothLewy body disease details
UrineDetected and Quantified4.211 +/- 5.162 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
 details
UrineDetected and Quantified0.0053 - 0.0143 umol/mmol creatinineAdult (>18 years old)BothADPKD details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
UrineDetected and Quantified0.13 +/- 0.04 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia		 details
UrineDetected and Quantified3.0408 +/- 2.1926 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
 details
UrineDetected and Quantified2.8234 +/- 1.7792 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Analysis of 30 no...
 details
Associated Disorders and Diseases
Disease References
Epilepsy
  1. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Alzheimer's disease
  1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
  2. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Heart failure
  1. Norrelund H, Wiggers H, Halbirk M, Frystyk J, Flyvbjerg A, Botker HE, Schmitz O, Jorgensen JO, Christiansen JS, Moller N: Abnormalities of whole body protein turnover, muscle metabolism and levels of metabolic hormones in patients with chronic heart failure. J Intern Med. 2006 Jul;260(1):11-21. [PubMed:16789974 ]
Maple syrup urine disease
  1. Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. [PubMed:6422161 ]
  2. Yunus ZM, Kamaludin DA, Mamat M, Choy YS, Ngu L: Clinical and biochemical profiles of maple syrup urine disease in malaysian children. JIMD Rep. 2012;5:99-107. doi: 10.1007/8904_2011_105. Epub 2011 Dec 11. [PubMed:23430924 ]
  3. Barschak AG, Marchesan C, Sitta A, Deon M, Giugliani R, Wajner M, Vargas CR: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem. 2008 Mar;41(4-5):317-24. Epub 2007 Dec 5. [PubMed:18088602 ]
  4. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
  5. Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.
Colorectal cancer
  1. Ritchie SA, Ahiahonu PW, Jayasinghe D, Heath D, Liu J, Lu Y, Jin W, Kavianpour A, Yamazaki Y, Khan AM, Hossain M, Su-Myat KK, Wood PL, Krenitsky K, Takemasa I, Miyake M, Sekimoto M, Monden M, Matsubara H, Nomura F, Goodenowe DB: Reduced levels of hydroxylated, polyunsaturated ultra long-chain fatty acids in the serum of colorectal cancer patients: implications for early screening and detection. BMC Med. 2010 Feb 15;8:13. doi: 10.1186/1741-7015-8-13. [PubMed:20156336 ]
  2. Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ]
  3. Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ]
  4. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  5. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  6. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Early preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
  2. Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ]
  3. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Saccharopinuria
  1. Simell O, Visakorpi JK, Donner M: Saccharopinuria. Arch Dis Child. 1972 Feb;47(251):52-5. [PubMed:5018656 ]
Dihydrolipoamide Dehydrogenase Deficiency
  1. Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Branched-chain Keto Acid Dehydrogenase Kinase Deficiency
  1. Novarino G, El-Fishawy P, Kayserili H, Meguid NA, Scott EM, Schroth J, Silhavy JL, Kara M, Khalil RO, Ben-Omran T, Ercan-Sencicek AG, Hashish AF, Sanders SJ, Gupta AR, Hashem HS, Matern D, Gabriel S, Sweetman L, Rahimi Y, Harris RA, State MW, Gleeson JG: Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy. Science. 2012 Oct 19;338(6105):394-7. doi: 10.1126/science.1224631. Epub 2012 Sep 6. [PubMed:22956686 ]
Lipoyltransferase 1 Deficiency
  1. Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
Leukemia
  1. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Schizophrenia
  1. Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [PubMed:7595563 ]
  2. Bjerkenstedt L, Edman G, Hagenfeldt L, Sedvall G, Wiesel FA: Plasma amino acids in relation to cerebrospinal fluid monoamine metabolites in schizophrenic patients and healthy controls. Br J Psychiatry. 1985 Sep;147:276-82. [PubMed:2415198 ]
  3. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Crohn's disease
  1. Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ]
  2. Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ]
  3. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
  1. Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ]
  2. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
  3. Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ]
  4. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Irritable bowel syndrome
  1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
  2. Hong YS, Hong KS, Park MH, Ahn YT, Lee JH, Huh CS, Lee J, Kim IK, Hwang GS, Kim JS: Metabonomic understanding of probiotic effects in humans with irritable bowel syndrome. J Clin Gastroenterol. 2011 May-Jun;45(5):415-25. doi: 10.1097/MCG.0b013e318207f76c. [PubMed:21494186 ]
Autism
  1. De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
Perillyl alcohol administration for cancer treatment
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Pancreatic cancer
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
  2. OuYang D, Xu J, Huang H, Chen Z: Metabolomic profiling of serum from human pancreatic cancer patients using 1H NMR spectroscopy and principal component analysis. Appl Biochem Biotechnol. 2011 Sep;165(1):148-54. doi: 10.1007/s12010-011-9240-0. Epub 2011 Apr 20. [PubMed:21505807 ]
  3. Zhang L, Jin H, Guo X, Yang Z, Zhao L, Tang S, Mo P, Wu K, Nie Y, Pan Y, Fan D: Distinguishing pancreatic cancer from chronic pancreatitis and healthy individuals by (1)H nuclear magnetic resonance-based metabonomic profiles. Clin Biochem. 2012 Sep;45(13-14):1064-9. doi: 10.1016/j.clinbiochem.2012.05.012. Epub 2012 May 19. [PubMed:22613268 ]
Periodontal disease
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Frontotemporal dementia
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Attachment loss
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Missing teeth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Autosomal dominant polycystic kidney disease
  1. Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDDB00167
Phenol Explorer Compound IDNot Available
FooDB IDFDB012397
KNApSAcK IDC00001374
Chemspider ID6067
KEGG Compound IDC00407
BioCyc IDILE
BiGG ID34887
Wikipedia LinkIsoleucine
METLIN ID5193
PubChem Compound6306
PDB IDNot Available
ChEBI ID17191
Food Biomarker OntologyNot Available
VMH IDILE_L
MarkerDB IDMDB00000083
Good Scents IDNot Available
References
Synthesis Reference Marvel, C. S. L-Isoleucine. Organic Syntheses (1941), 21 60-4.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
  2. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
  3. Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
  4. Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
  5. Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [PubMed:6198473 ]
  6. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
  7. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
  8. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
  9. He XY, Yang SY: Roles of type 10 17beta-hydroxysteroid dehydrogenase in intracrinology and metabolism of isoleucine and fatty acids. Endocr Metab Immune Disord Drug Targets. 2006 Mar;6(1):95-102. [PubMed:16611167 ]
  10. Vaalasti A, Suomalainen H, Kuokkanen K, Rechardt L: Neuropeptides in cutaneous neurofibromas of von Recklinghausen's disease. J Cutan Pathol. 1990 Dec;17(6):371-3. [PubMed:1981573 ]
  11. Eriste E, Norberg A, Bonetto V, Nepomuceno D, Lovenberg TW, Sillard R, Jornvall H: A C-terminally elongated form of PHI from porcine intestine. Cell Mol Life Sci. 1999 Nov 15;56(7-8):709-13. [PubMed:11212317 ]
  12. Jalan R, Olde Damink SW, Lui HF, Glabus M, Deutz NE, Hayes PC, Ebmeier K: Oral amino acid load mimicking hemoglobin results in reduced regional cerebral perfusion and deterioration in memory tests in patients with cirrhosis of the liver. Metab Brain Dis. 2003 Mar;18(1):37-49. [PubMed:12603081 ]
  13. Hervieu G, Segretain D, Nahon JL: Developmental and stage-dependent expression of melanin-concentrating hormone in mammalian germ cells. Biol Reprod. 1996 Jun;54(6):1161-72. [PubMed:8724342 ]
  14. Suk FM, Lin MH, Newman M, Pan S, Chen SH, Liu JD, Shih C: Replication advantage and host factor-independent phenotypes attributable to a common naturally occurring capsid mutation (I97L) in human hepatitis B virus. J Virol. 2002 Dec;76(23):12069-77. [PubMed:12414948 ]
  15. De Miranda J, Panizzutti R, Foltyn VN, Wolosker H: Cofactors of serine racemase that physiologically stimulate the synthesis of the N-methyl-D-aspartate (NMDA) receptor coagonist D-serine. Proc Natl Acad Sci U S A. 2002 Oct 29;99(22):14542-7. Epub 2002 Oct 22. [PubMed:12393813 ]
  16. Blomstrand E, Eliasson J, Karlsson HK, Kohnke R: Branched-chain amino acids activate key enzymes in protein synthesis after physical exercise. J Nutr. 2006 Jan;136(1 Suppl):269S-73S. [PubMed:16365096 ]
  17. Sato T, Shimada Y, Nagasawa N, Nakanishi S, Jingami H: Amino acid mutagenesis of the ligand binding site and the dimer interface of the metabotropic glutamate receptor 1. Identification of crucial residues for setting the activated state. J Biol Chem. 2003 Feb 7;278(6):4314-21. Epub 2002 Nov 19. [PubMed:12444084 ]
  18. Edvinsson L: Innervation and effects of dilatory neuropeptides on cerebral vessels. New aspects. Blood Vessels. 1991;28(1-3):35-45. [PubMed:2001478 ]
  19. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]
  20. Allahwala A, Ahmed S, Afroze B: Maple syrup urine disease: magnetic resonance imaging findings in three patients. J Pak Med Assoc. 2021 Apr;71(4):1309-1313. doi: 10.47391/JPMA.1341. [PubMed:34125801 ]
  21. Lynch CJ, Adams SH: Branched-chain amino acids in metabolic signalling and insulin resistance. Nat Rev Endocrinol. 2014 Dec;10(12):723-36. doi: 10.1038/nrendo.2014.171. Epub 2014 Oct 7. [PubMed:25287287 ]
  22. Xiao F, Yu J, Guo Y, Deng J, Li K, Du Y, Chen S, Zhu J, Sheng H, Guo F: Effects of individual branched-chain amino acids deprivation on insulin sensitivity and glucose metabolism in mice. Metabolism. 2014 Jun;63(6):841-50. doi: 10.1016/j.metabol.2014.03.006. Epub 2014 Mar 15. [PubMed:24684822 ]

Enzymes

Enzyme Details
1. Branched-chain-amino-acid aminotransferase, cytosolic
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular weight:
38644.77
Reactions
Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + Glutamic aciddetails
References
  1. Goto M, Miyahara I, Hayashi H, Kagamiyama H, Hirotsu K: Crystal structures of branched-chain amino acid aminotransferase complexed with glutamate and glutarate: true reaction intermediate and double substrate recognition of the enzyme. Biochemistry. 2003 Apr 8;42(13):3725-33. [PubMed:12667063 ]
  2. Chen CD, Huang TF, Lin CH, Guan HH, Hsieh YC, Lin YH, Huang YC, Liu MY, Chang WC, Chen CJ: Purification, crystallization and preliminary X-ray crystallographic analysis of branched-chain aminotransferase from Deinococcus radiodurans. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2007 Jun 1;63(Pt 6):492-4. Epub 2007 May 5. [PubMed:17554170 ]
  3. Beck HC: Branched-chain fatty acid biosynthesis in a branched-chain amino acid aminotransferase mutant of Staphylococcus carnosus. FEMS Microbiol Lett. 2005 Feb 1;243(1):37-44. [PubMed:15667998 ]
  4. Thage BV, Rattray FP, Laustsen MW, Ardo Y, Barkholt V, Houlberg U: Purification and characterization of a branched-chain amino acid aminotransferase from Lactobacillus paracasei subsp. paracasei CHCC 2115. J Appl Microbiol. 2004;96(3):593-602. [PubMed:14962140 ]
  5. Madsen SM, Beck HC, Ravn P, Vrang A, Hansen AM, Israelsen H: Cloning and inactivation of a branched-chain-amino-acid aminotransferase gene from Staphylococcus carnosus and characterization of the enzyme. Appl Environ Microbiol. 2002 Aug;68(8):4007-14. [PubMed:12147502 ]
Enzyme Details
2. Branched-chain-amino-acid aminotransferase, mitochondrial
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:
BCAT2
Uniprot ID:
O15382
Molecular weight:
33776.315
Reactions
Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + Glutamic aciddetails
References
  1. Berger BJ, English S, Chan G, Knodel MH: Methionine regeneration and aminotransferases in Bacillus subtilis, Bacillus cereus, and Bacillus anthracis. J Bacteriol. 2003 Apr;185(8):2418-31. [PubMed:12670965 ]
Enzyme Details
3. Isoleucine--tRNA ligase, cytoplasmic
General function:
Involved in nucleotide binding
Specific function:
Not Available
Gene Name:
IARS
Uniprot ID:
P41252
Molecular weight:
144496.915
Reactions
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-isoleucyl-tRNA(Ile)details
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-Isoleucyl-tRNA(Ile)details
References
  1. Crasto CF, Forrest AK, Karoli T, March DR, Mensah L, O'Hanlon PJ, Nairn MR, Oldham MD, Yue W, Banwell MG, Easton CJ: Synthesis and activity of analogues of the isoleucyl tRNA synthetase inhibitor SB-203207. Bioorg Med Chem. 2003 Jul 3;11(13):2687-94. [PubMed:12788342 ]
  2. Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. [PubMed:12783542 ]
  3. Mock ML, Michon T, van Hest JC, Tirrell DA: Stereoselective incorporation of an unsaturated isoleucine analogue into a protein expressed in E. coli. Chembiochem. 2006 Jan;7(1):83-7. [PubMed:16397872 ]
  4. Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. [PubMed:14672940 ]
  5. Pezo V, Metzgar D, Hendrickson TL, Waas WF, Hazebrouck S, Doring V, Marliere P, Schimmel P, De Crecy-Lagard V: Artificially ambiguous genetic code confers growth yield advantage. Proc Natl Acad Sci U S A. 2004 Jun 8;101(23):8593-7. Epub 2004 May 26. [PubMed:15163798 ]
Enzyme Details
4. Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
Gene Name:
ACADSB
Uniprot ID:
P45954
Molecular weight:
47485.035
References
  1. Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [PubMed:15615815 ]
  2. Madsen PP, Kibaek M, Roca X, Sachidanandam R, Krainer AR, Christensen E, Steiner RD, Gibson KM, Corydon TJ, Knudsen I, Wanders RJ, Ruiter JP, Gregersen N, Andresen BS: Short/branched-chain acyl-CoA dehydrogenase deficiency due to an IVS3+3A>G mutation that causes exon skipping. Hum Genet. 2006 Feb;118(6):680-90. Epub 2005 Nov 30. [PubMed:16317551 ]
  3. Korman SH: Inborn errors of isoleucine degradation: a review. Mol Genet Metab. 2006 Dec;89(4):289-99. Epub 2006 Sep 6. [PubMed:16950638 ]
Enzyme Details
5. L-amino-acid oxidase
General function:
Involved in oxidoreductase activity
Specific function:
Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
Gene Name:
IL4I1
Uniprot ID:
Q96RQ9
Molecular weight:
65327.26
Reactions
Isoleucine + Water + Oxygen → 3-Methyl-2-oxovaleric acid + Ammonia + Hydrogen peroxidedetails
Enzyme Details
6. Isoleucine--tRNA ligase, mitochondrial
General function:
Involved in nucleotide binding
Specific function:
Not Available
Gene Name:
IARS2
Uniprot ID:
Q9NSE4
Molecular weight:
113790.565
Reactions
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-isoleucyl-tRNA(Ile)details
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-Isoleucyl-tRNA(Ile)details
References
  1. Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. [PubMed:12783542 ]
  2. Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. [PubMed:14672940 ]
  3. Zhu B, Zhao MW, Eriani G, Wang ED: A present-day aminoacyl-tRNA synthetase with ancestral editing properties. RNA. 2007 Jan;13(1):15-21. Epub 2006 Nov 9. [PubMed:17095543 ]
  4. Fukunaga R, Yokoyama S: Crystal structure of leucyl-tRNA synthetase from the archaeon Pyrococcus horikoshii reveals a novel editing domain orientation. J Mol Biol. 2005 Feb 11;346(1):57-71. Epub 2004 Dec 19. [PubMed:15663927 ]
  5. Fukunaga R, Yokoyama S: Structural basis for substrate recognition by the editing domain of isoleucyl-tRNA synthetase. J Mol Biol. 2006 Jun 16;359(4):901-12. Epub 2006 Apr 25. [PubMed:16697013 ]

Transporters

Enzyme Details
1. Monocarboxylate transporter 10
General function:
Involved in transmembrane transport
Specific function:
Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:
SLC16A10
Uniprot ID:
Q8TF71
Molecular weight:
55492.1
References
  1. Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508 ]