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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2022-03-07 02:49:05 UTC
HMDB IDHMDB0000687
Secondary Accession Numbers
  • HMDB00687
Metabolite Identification
Common NameL-Leucine
DescriptionLeucine (Leu) or L-leucine 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-leucine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Leucine 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. Leucine is essential in humans, meaning the body cannot synthesize it, and it must be obtained from the diet. Human dietary sources are foods that contain protein, such as meats, dairy products, soy products, beans and legumes. L-Leucine is a branched chain amino acid (BCAA). The BCAAs consist of leucine, valine and isoleucine (and occasionally threonine). BCAAs are essential amino acids whose carbon structure is marked by a branch point at the beta-carbon position. BCAAs are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. 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. The different metabolism 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 respectively. The primary metabolic end products of leucine metabolism are acetyl-CoA and acetoacetate; consequently, it is one of the two exclusively ketogenic amino acids, with lysine being the other. Leucine is the most important ketogenic amino acid in humans. The vast majority of l-leucine metabolism is initially catalyzed by the branched-chain amino acid aminotransferase enzyme, producing alpha-ketoisocaproate (alpha-KIC). alpha-KIC is metabolized by the mitochondrial enzyme branched-chain alpha-ketoacid dehydrogenase, which converts it to isovaleryl-CoA. Isovaleryl-CoA is subsequently metabolized by the enzyme isovaleryl-CoA dehydrogenase and converted to beta-methylcrotonyl-CoA (MC-CoA), which is used in the synthesis of acetyl-CoA and other compounds. During biotin deficiency, HMB can be synthesized from MC-CoA via enoyl-CoA hydratase and an unknown thioesterase enzyme, which convert MC-CoA into HMB-CoA and HMB-CoA into HMB respectively. Leucine has the capacity to directly stimulate myofibrillar muscle protein synthesis (PMID 15051860 ). This effect of leucine arises results from its role as an activator of the mechanistic target of rapamycin (mTOR) (PMID 23551944 ) a serine-threonine protein kinase that regulates protein biosynthesis and cell growth. The activation of mTOR by leucine is mediated through Rag GTPases. Leucine, like other BCAAs, is associated with insulin resistance. In particular, higher levels of leucine are observed in the blood of diabetic mice, rats, and humans (PMID 25287287 ). BCAAs such as leucine have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. Persistently low leucine levels can result in decreased appetite, poor feeding, lethargy, poor growth, weight loss, skin rashes, hair loss, and desquamation. Many types of inborn errors of BCAA metabolism exist and these are marked by various abnormalities. The most common form is maple syrup urine disease, marked by a characteristic urinary odor. Other abnormalities are associated with a wide range of symptoms, such as mental retardation, ataxia, hypoglycemia, spinal muscle atrophy, rash, vomiting and excessive muscle movement. Most forms of BCAA metabolism errors are corrected by dietary restriction of BCAAs and at least one form is correctable by supplementation with 10 mg of biotin daily. - BCAAs are useful because they are metabolized primarily by muscle. Stress states - e.g surgery, trauma, cirrhosis, infections, fever and starvation--require proportionately more BCAAs than other amino acids and probably proportionately more leucine than either valine or isoleucine. BCAAs and other amino acids are frequently fed intravenously (TPN) to malnourished surgical patients and in some cases of severe trauma. BCAAs, particularly leucine, stimulate protein synthesis, increase reutilization of amino acids in many organs and reduce protein breakdown. Furthermore, leucine can be an important source of calories, and is superior as fuel to the ubiquitous intravenous glucose (dextrose). - Leucine also stimulates insulin release, which in turn stimulates protein synthesis and inhibits protein breakdown. These effects are particularly useful in athletic training. Huntington's chorea and anorexic disorders both are characterized by low serum BCAAs. These diseases, as well as forms of Parkinson's, may respond to BCAA therapy.
Structure
Data?1582752149
Synonyms
ValueSource
(2S)-2-Amino-4-methylpentanoic acidChEBI
(2S)-alpha-2-Amino-4-methylvaleric acidChEBI
(2S)-alpha-LeucineChEBI
(S)-(+)-LeucineChEBI
(S)-LeucineChEBI
2-Amino-4-methylvaleric acidChEBI
LChEBI
L-LeucinChEBI
L-LeuzinChEBI
LeuChEBI
LEUCINEChEBI
(2S)-2-Amino-4-methylpentanoateGenerator
(2S)-a-2-Amino-4-methylvalerateGenerator
(2S)-a-2-Amino-4-methylvaleric acidGenerator
(2S)-alpha-2-Amino-4-methylvalerateGenerator
(2S)-Α-2-amino-4-methylvalerateGenerator
(2S)-Α-2-amino-4-methylvaleric acidGenerator
(2S)-a-LeucineGenerator
(2S)-Α-leucineGenerator
2-Amino-4-methylvalerateGenerator
(S)-2-Amino-4-methylpentanoateHMDB
(S)-2-Amino-4-methylpentanoic acidHMDB
(S)-2-Amino-4-methylvalerateHMDB
(S)-2-Amino-4-methylvaleric acidHMDB
4-Methyl-L-norvalineHMDB
L-(+)-LeucineHMDB
L-a-AminoisocaproateHMDB
L-a-Aminoisocaproic acidHMDB
L-alpha-AminoisocaproateHMDB
L-alpha-Aminoisocaproic acidHMDB
Leucine, L-isomerHMDB
L-Isomer leucineHMDB
Leucine, L isomerHMDB
Chemical FormulaC6H13NO2
Average Molecular Weight131.1729
Monoisotopic Molecular Weight131.094628665
IUPAC Name(2S)-2-amino-4-methylpentanoic acid
Traditional NameL-leucine
CAS Registry Number61-90-5
SMILES
CC(C)C[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C6H13NO2/c1-4(2)3-5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t5-/m0/s1
InChI KeyROHFNLRQFUQHCH-YFKPBYRVSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as leucine and derivatives. Leucine and derivatives are compounds containing leucine or a derivative thereof resulting from reaction of leucine 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 ParentLeucine and derivatives
Alternative Parents
Substituents
  • Leucine 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

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point268 - 288 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility21.5 mg/mLNot Available
LogP-1.52HANSCH,C ET AL. (1995)
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Baker133.8930932474
[M-H]-McLean131.530932474
[M-H]-MetCCS_test_neg128.530932474
[M-H]-Astarita_neg128.730932474
[M+H]+Astarita_pos128.330932474
[M+H]+Baker136.5430932474
[M+H]+McLean134.730932474
[M-H]-Not Available132.6http://allccs.zhulab.cn/database/detail?ID=AllCCS00000088
[M+H]+Not Available134.9http://allccs.zhulab.cn/database/detail?ID=AllCCS00000088
Predicted Molecular Properties
PropertyValueSource
Water Solubility69.8 g/LALOGPS
logP-1.8ALOGPS
logP-1.6ChemAxon
logS-0.27ALOGPS
pKa (Strongest Acidic)2.79ChemAxon
pKa (Strongest Basic)9.52ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.17 m³·mol⁻¹ChemAxon
Polarizability14.16 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]+129.24231661259
DarkChem[M-H]-124.62831661259
AllCCS[M+H]+132.01732859911
AllCCS[M-H]-130.0332859911
DeepCCS[M+H]+129.79730932474
DeepCCS[M-H]-126.15230932474
DeepCCS[M-2H]-163.52130932474
DeepCCS[M+Na]+138.67430932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
L-LeucineCC(C)C[C@H](N)C(O)=O1933.5Standard polar33892256
L-LeucineCC(C)C[C@H](N)C(O)=O1111.9Standard non polar33892256
L-LeucineCC(C)C[C@H](N)C(O)=O1204.1Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
L-Leucine,1TMS,isomer #1CC(C)C[C@H](N)C(=O)O[Si](C)(C)C1170.2Semi standard non polar33892256
L-Leucine,1TMS,isomer #2CC(C)C[C@H](N[Si](C)(C)C)C(=O)O1254.8Semi standard non polar33892256
L-Leucine,2TMS,isomer #1CC(C)C[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1291.2Semi standard non polar33892256
L-Leucine,2TMS,isomer #1CC(C)C[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1309.8Standard non polar33892256
L-Leucine,2TMS,isomer #1CC(C)C[C@H](N[Si](C)(C)C)C(=O)O[Si](C)(C)C1399.1Standard polar33892256
L-Leucine,2TMS,isomer #2CC(C)C[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1455.3Semi standard non polar33892256
L-Leucine,2TMS,isomer #2CC(C)C[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1364.0Standard non polar33892256
L-Leucine,2TMS,isomer #2CC(C)C[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1562.8Standard polar33892256
L-Leucine,3TMS,isomer #1CC(C)C[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1493.4Semi standard non polar33892256
L-Leucine,3TMS,isomer #1CC(C)C[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1445.1Standard non polar33892256
L-Leucine,3TMS,isomer #1CC(C)C[C@@H](C(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1394.1Standard polar33892256
L-Leucine,1TBDMS,isomer #1CC(C)C[C@H](N)C(=O)O[Si](C)(C)C(C)(C)C1398.1Semi standard non polar33892256
L-Leucine,1TBDMS,isomer #2CC(C)C[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O1488.3Semi standard non polar33892256
L-Leucine,2TBDMS,isomer #1CC(C)C[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1716.4Semi standard non polar33892256
L-Leucine,2TBDMS,isomer #1CC(C)C[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1724.8Standard non polar33892256
L-Leucine,2TBDMS,isomer #1CC(C)C[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1705.1Standard polar33892256
L-Leucine,2TBDMS,isomer #2CC(C)C[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1852.9Semi standard non polar33892256
L-Leucine,2TBDMS,isomer #2CC(C)C[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1787.1Standard non polar33892256
L-Leucine,2TBDMS,isomer #2CC(C)C[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1780.4Standard polar33892256
L-Leucine,3TBDMS,isomer #1CC(C)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)C2117.0Semi standard non polar33892256
L-Leucine,3TBDMS,isomer #1CC(C)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)C2059.0Standard non polar33892256
L-Leucine,3TBDMS,isomer #1CC(C)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)C1820.9Standard polar33892256
Spectra
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • Adipose Tissue
  • Adrenal Medulla
  • Bladder
  • Epidermis
  • Fibroblasts
  • Intestine
  • Kidney
  • Neuron
  • Placenta
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Testis
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified70.00-180.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified70.0 +/- 25.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified134.0 +/- 20.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified160.0 +/- 27.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified157.0 +/- 30.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified55-183 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified43-165 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified40-180 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified55-174 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified158.3 +/- 37.6 uMChildren (1 - 13 years old)Not Specified
Normal
details
BloodDetected and Quantified156 (134-183) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified48-160 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified68-168 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified159 +/- 23 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified76-168 uMAdolescent (13-18 years old)Not Specified
Normal
details
BloodDetected and Quantified49-216 uMChildren (1-13 years old)Not Specified
Normal
details
BloodDetected and Quantified120 +/- 11 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified168 (134-216) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected and Quantified72-201 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified64-164 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified150.1 +/- 27.7 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified127.4(100.5-150.2) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified123 (98-148) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified251 +/- 5 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified98.7 +/- 11.5 uMAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified24.4 +/- 6.3 uMAdult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified16 +/- 9 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified10.1 +/- 2.1 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified3.3-21.3 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified1.4-23.6 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified14.5 +/- 3.7 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified11.0-18.7 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified14.1-22.9 uMNewborn (0-30 days old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified18.6 +/- 4.1 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified11.2 +/- 3.5 uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified8209 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
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 (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothNormal 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 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)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected and Quantified50.773 +/- 61.369 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 and Quantified2014.898 +/- 1413.402 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified1780 +/- 1160 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified2076 +/- 837 nmol/g wet fecesAdult (>18 years old)Female
Normal
details
FecesDetected and Quantified300 +/- 290 nmol/g wet fecesAdult (>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)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 QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
FecesDetected and Quantified7601 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
FecesDetected and Quantified7298 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
SalivaDetected and Quantified6.61 +/- 4.84 uMAdult (>18 years old)Both
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified16.0 +/- 21.5 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected and Quantified8.1 +/- 4.1 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified2.79 +/- 1.21 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
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 Quantified11.3 +/- 7.6 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)Not SpecifiedNormal details
SalivaDetected and Quantified4.2 +/- 3.6 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified53.50 +/- 32.95 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified3.80 +/- 2.10 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified6.7 +/- 5.8 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified7.24 +/- 5.62 uMAdult (>18 years old)FemaleNormal details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
SweatDetected but not QuantifiedNot QuantifiedAdult BothNormal details
UrineDetected and Quantified2.04 - 12.44 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.24 - 8.37 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.24 - 8.37 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified13-40 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified28.27 +/- 11.31 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified44.09 +/- 22.61 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified44.09 +/- 24.87 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified7.547 +/- 4.945 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected and Quantified1.47-8.26 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified3.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.33-13.41 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified5.4 +/- 2.86 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified<7.013 umol/mmol creatinineAdolescent (13-18 years old)Not SpecifiedNormal details
UrineDetected and Quantified6.26 +/- 2.87 umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.4-2.3 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified6.10-19.07 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified7.58 +/- 4.10 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified4.868 (1.842-8.421) umol/mmol creatinineAdult (>18 years old)BothNormal
      Not Available
details
UrineDetected and Quantified<5.77 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.15-19.93 umol/mmol creatinineNewborn (0-30 days old)Female
Normal
details
UrineDetected and Quantified0.45-17.10 umol/mmol creatinineNewborn (0-30 days old)Male
Normal
details
UrineDetected and Quantified4.030 +/- 4.10 umol/mmol creatinineNewborn (0-30 days old)Male
Normal
details
UrineDetected and Quantified7.37 +/- 5.63 umol/mmol creatinineChildren (0-2 years old)Male
Normal
details
UrineDetected and Quantified7.46 +/- 5.15 umol/mmol creatinineChildren (0-2 years old)Female
Normal
details
UrineDetected and Quantified8.29 +/- 6.53 umol/mmol creatinineNewborn (0-30 days old)Female
Normal
details
UrineDetected and Quantified<22.62 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified6.67 (0-13.33) umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified2.5 +/- 1.0 umol/mmol creatinineChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified3.0+/- 1.5 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified2.5 +/- 1.3 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified0.6 +/- 0.2 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified3.0 (1.6-6.0) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.6 +/- 0.3 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
details
UrineDetected and Quantified0.6 +/-0.3 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
details
UrineDetected and Quantified3.166-45.450 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified2.8 (1.6-5.4) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.70-18.89 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified1.4-2.3 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified1.2 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified8.0076 +/- 3.0528 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected and Quantified1.36-11.31 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified2.04 - 19.67 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.83 - 9.27 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.83 - 12.44 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.04 - 13.23 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.83 - 14.02 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified67.3 (34.6) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified60.0 (46.9) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified74.8 +/- 34.3 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified1694 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
details
BloodDetected and Quantified66.0 - 161.0 uMNewborn (0-30 days old)BothPhenylketonuria details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified72.95 +/- 24.0 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18
details
BloodDetected and Quantified71.6 +/- 40.6 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified66 uMInfant (0-1 year old)MaleFumaric aciduria details
BloodDetected and Quantified365.9 +/- 287.9 uMChildren (1 - 13 years old)Not Specified
Maple syrup urine disease
details
BloodDetected and Quantified67.5 (15.5) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified96.9 (74.9) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified67.5 (15-76) uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
BloodDetected and Quantified171 +/- 30 uMAdult (>18 years old)MaleSchizophrenia details
BloodDetected and Quantified141 +/- 26 uMAdult (>18 years old)FemaleSchizophrenia details
BloodDetected and Quantified686.117 uMInfant (0-1 year old)MaleDihydrolipoamide Dehydrogenase Deficiency details
BloodDetected and Quantified18-46 uMAdolescent (13-18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified20-46 uMAdult (>18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified15-113 uMChildren (1-13 years old)BothBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified138.2(116.4-167.3) uMChildren (1-13 uears old)Both
Environmental enteric dysfunction
details
BloodDetected and Quantified73.74 (23.04) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified69.55 (16.39) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified114.5 (98.5) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
BloodDetected and Quantified87.1 (61.9) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified95.3 (89.3-100.0) uMAdult (>18 years old)Both
Epilepsy
details
BloodDetected and Quantified273.0 +/- 10.0 uMAdult (>18 years old)Both
Heart failure
details
BloodDetected and Quantified103.0 (97.8-108.0) uMAdult (>18 years old)Both
Epilepsy
details
BloodDetected and Quantified643.0 - 667.0 uMNewborn (0-30 days old)BothMaple syrup urine disease (MSUD) details
Cerebrospinal Fluid (CSF)Detected and Quantified892 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
details
Cerebrospinal Fluid (CSF)Detected and Quantified11.5 +/- 1.5 uMAdult (>18 years old)BothAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified26.7 uMNewborn (0-30 days old)FemaleEpilepsy, early-onset, vitamin B6-dependent details
Cerebrospinal Fluid (CSF)Detected and Quantified16.5 +/- 7.0 uMChildren (1-13 years old)Not Specified
Leukemia
details
Cerebrospinal Fluid (CSF)Detected and Quantified8.2 uMChildren (1-13 years old)FemaleEpilepsy, early-onset, vitamin B6-dependent details
Cerebrospinal Fluid (CSF)Detected and Quantified13.1 +/- 5.6 uMChildren (1-13 years old)Not SpecifiedLeukemia 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)BothImmunoglobulin A nephropathy (IgAN) progressor details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothAutism details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Femaleankylosing spondylitis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Femalerheumatoid arthritis 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 QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal 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)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal 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)BothColorectal Cancer 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
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
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 Quantified2.73 +/- 0.76 uMAdult (>18 years old)MaleAlzheimer's disease 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 squamous cell carcinoma (OSCC) details
SalivaDetected and Quantified3.74 +/- 0.65 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral leukoplakia (OLK) details
SalivaDetected and Quantified5.66 +/- 3.83 uMAdult (>18 years old)BothLewy body disease details
UrineDetected and Quantified8.333 +/- 7.338 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
UrineDetected and Quantified53.900-17.750 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedFumaric aciduria details
UrineDetected and Quantified0.91 +/- 0.11 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
UrineDetected and Quantified6.3164 +/- 4.2963 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected and Quantified5.6052 +/- 3.0948 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 ]
Maple syrup urine disease
  1. Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
  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 ]
  3. 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.
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 ]
Phenylketonuria
  1. Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
Schizophrenia
  1. 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 ]
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 preecl