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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:44:43 UTC
HMDB IDHMDB0000557
Secondary Accession Numbers
  • HMDB0002291
  • HMDB00557
  • HMDB02291
Metabolite Identification
Common NameL-Alloisoleucine
DescriptionL-alloisoleucine is a branched chain amino acid and is a stereo-isomer of L-isoleucine. It is a common constituent of human plasma (albeit at low levels). L-alloisoleucine is produced as a byproduct of isoleucine transamination. L-Isoleucine is one of the four common amino acids (the 3 others being threonine, hydroxyproline, and hydroxylysine) that have two asymmetric carbon atoms which produce four structural possibilities for the same chemical composition. L-alloisoleucine differs from L-isoleucine by having having different stereochemistry around its beta carbon. Plasma L-alloisoleucine, which is derived from L-isoleucine in vivo, can be used for the diagnosis of maple syrup urine disease (MSUD), a genetic disorder. Indeed, plasma L-alloisoleucine levels above 5 umol/L is the most specific and most sensitive diagnostic marker for all forms of MSUD (PMID: 10508118 ). In MSUD the degradation of the essential branched-chain L-amino acids leucine, valine, and isoleucine and their derived 2-oxoacids is impaired because of an inherited deficiency in branched-chain 2-oxoacid dehydrogenase complex (EC 1.2.4.4) activity. The accumulation of branched-chain compounds in blood and other body fluids can exert neurotoxic effects.
Structure
Data?1582752140
Synonyms
ValueSource
(2S,3R)-2-Amino-3-methylpentanoic acidChEBI
AlleChEBI
Allo-L-isoleucineChEBI
L(+)-AlloisoleucineChEBI
Threo-3-methyl-L-norvalineChEBI
Threo-L-isoleucineChEBI
(2S,3R)-2-Amino-3-methylpentanoateGenerator
AlloisoleucineHMDB
IsoleucineHMDB
Isoleucine, L isomerHMDB
Isoleucine, L-isomerHMDB
L-IsoleucineHMDB
L-Isomer isoleucineHMDB
(3R)-LS-IsoleucineHMDB
2-Amino-3-methyl-[S-(r*,s*)]-pentanoateHMDB
2-Amino-3-methyl-[S-(r*,s*)]-pentanoic acidHMDB
Allo-isoleucineHMDB
L-Allo-isoleucineHMDB
[S-(R*,s*)]-2-amino-3-methylpentanoateHMDB
[S-(R*,s*)]-2-amino-3-methylpentanoic acidHMDB
Chemical FormulaC6H13NO2
Average Molecular Weight131.1729
Monoisotopic Molecular Weight131.094628665
IUPAC Name(2S,3R)-2-amino-3-methylpentanoic acid
Traditional Nameallo-isoleucine
CAS Registry Number1509-34-8
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+/m1/s1
InChI KeyAGPKZVBTJJNPAG-UHNVWZDZSA-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

Adverse health effect

Disposition

Biological location

Source

Route of exposure

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point60 - 64 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility34.4 mg/mL at 25 °CNot Available
LogP-1.70HANSCH,C ET AL. (1995)
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Not Available134.452http://allccs.zhulab.cn/database/detail?ID=AllCCS00001745
Predicted Molecular Properties
PropertyValueSource
Water Solubility114 g/LALOGPS
logP10(-1.7) g/LALOGPS
logP10(-1.5) g/LChemAxon
logS10(-0.06) g/LALOGPS
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
Polarizability14.23 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]+128.07631661259
DarkChem[M-H]-125.35731661259
AllCCS[M+H]+132.34232859911
AllCCS[M-H]-129.05832859911

Predicted Kovats Retention Indices

Underivatized

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

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
L-Alloisoleucine,1TMS,isomer #1CC[C@@H](C)[C@H](N)C(=O)O[Si](C)(C)C1180.4Semi standard non polar33892256
L-Alloisoleucine,1TMS,isomer #2CC[C@@H](C)[C@H](N[Si](C)(C)C)C(=O)O1292.1Semi standard non polar33892256
L-Alloisoleucine,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-Alloisoleucine,2TMS,isomer #1CC[C@@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1308.4Standard non polar33892256
L-Alloisoleucine,2TMS,isomer #1CC[C@@H](C)[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1419.5Standard polar33892256
L-Alloisoleucine,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-Alloisoleucine,2TMS,isomer #2CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1322.9Standard non polar33892256
L-Alloisoleucine,2TMS,isomer #2CC[C@@H](C)[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1511.7Standard polar33892256
L-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,1TBDMS,isomer #1CC[C@@H](C)[C@H](N)C(=O)O[Si](C)(C)C(C)(C)C1396.9Semi standard non polar33892256
L-Alloisoleucine,1TBDMS,isomer #2CC[C@@H](C)[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O1519.3Semi standard non polar33892256
L-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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-Alloisoleucine,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
Predicted GC-MSPredicted GC-MS Spectrum - L-Alloisoleucine GC-MS (Non-derivatized) - 70eV, Positivesplash10-00b9-9100000000-40bc9ef43da5f18be8832017-08-28Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Alloisoleucine GC-MS (1 TMS) - 70eV, Positivesplash10-00fr-9400000000-3a446aac1ca67e24d0d52017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Alloisoleucine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Alloisoleucine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Alloisoleucine GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Alloisoleucine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Alloisoleucine GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-001i-3900000000-703d0f350ebca58a7eb12012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-00ko-9000000000-b7c643e18a4283fc88e42012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-052f-9000000000-6b799d2abf4132bac9742012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 10V, Positive-QTOFsplash10-000x-9700000000-fd99c6b1f756eda9c88a2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 20V, Positive-QTOFsplash10-0006-9100000000-59856e2f5d49bcd1ddcc2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 40V, Positive-QTOFsplash10-0006-9000000000-a7cba7696438bbc753672021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 40V, Positive-QTOFsplash10-0a4i-9000000000-3f3a1371dbb8cee999752021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 20V, Negative-QTOFsplash10-0002-9000000000-b869312a540376f316612021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 40V, Negative-QTOFsplash10-03dl-9300000000-cc3611bb367101c1f9e02021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 10V, Positive-QTOFsplash10-0a4i-9100000000-f44a7527300b2ee92bd22021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 20V, Positive-QTOFsplash10-0a4i-9000000000-bb99da9182449d9406052021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Alloisoleucine 10V, Negative-QTOFsplash10-001i-1900000000-71d0a2db50a0c84ca4d42021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 10V, Positive-QTOFsplash10-0019-9500000000-00268b2694cde281641b2017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 20V, Positive-QTOFsplash10-000i-9000000000-4014793e17e0290790a42017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 40V, Positive-QTOFsplash10-0a4i-9000000000-eb9a97a14461e524e76e2017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 10V, Negative-QTOFsplash10-001i-2900000000-ead56ef2118d091489652017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 20V, Negative-QTOFsplash10-001i-5900000000-9335d1d91071c615ade62017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 40V, Negative-QTOFsplash10-0ab9-9000000000-e7df49772ae8a5d6660c2017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 10V, Positive-QTOFsplash10-000i-9100000000-6deeb9c0f6e2a54158ea2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 20V, Positive-QTOFsplash10-014r-9000000000-4fcbf47ecbe7cea12b862021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 40V, Positive-QTOFsplash10-0a4i-9000000000-175f823a38c66789e1302021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 10V, Negative-QTOFsplash10-001i-0900000000-06466c9190b13de8e4be2021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 20V, Negative-QTOFsplash10-001i-0900000000-06466c9190b13de8e4be2021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - L-Alloisoleucine 40V, Negative-QTOFsplash10-05al-9500000000-8575fe92309b181021412021-09-23Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0-4 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-4 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-4 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-4 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified<2.100 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified1.9 +/- 0.6 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.6 +/- 0.4 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0-0.5 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-0.5 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedBoth
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified<0.90 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified<0.90 umol/mmol creatinineAdolescent (13-18 years old)Not SpecifiedNormal details
UrineDetected and Quantified<0.79 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified<3.28 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified<1.13 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified0 - 0.11 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.11 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.68 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified<0.90 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified25.6 +/- 14.0 uMChildren (1 - 13 years old)Not Specified
Maple syrup urine disease
details
BloodDetected and Quantified127 +/- 1 uMChildren (1-13 years old)BothMaple syrup urine disease (MSUD) details
BloodDetected and Quantified1.500-2.600 uMNewborn (0-30 days old)Not Specified
2-Methylbutyryl-CoA dehydrogenase deficiency (SBACDD)
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
UrineDetected and Quantified1.524 +/- 0.425 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
  1. Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U: Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Clin Chem. 1999 Oct;45(10):1734-40. [PubMed:10508118 ]
  2. 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 ]
Short/branched chain acyl-CoA dehydrogenase deficiency
  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 ]
Colorectal cancer
  1. 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 ]
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
  • 248600 (Maple syrup urine disease)
  • 610006 (Short/branched chain acyl-CoA dehydrogenase deficiency)
  • 114500 (Colorectal cancer)
  • 610247 (Eosinophilic esophagitis)
DrugBank IDDB01739
Phenol Explorer Compound IDNot Available
FooDB IDFDB022117
KNApSAcK IDNot Available
Chemspider ID89698
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound99288
PDB IDNot Available
ChEBI ID43433
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000187
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General 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. Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U: Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Clin Chem. 1999 Oct;45(10):1734-40. [PubMed:10508118 ]