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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-10-13 21:55:16 UTC
HMDB IDHMDB00687
Secondary Accession NumbersNone
Metabolite Identification
Common NameL-Leucine
DescriptionBranched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids 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. 'BCAA' denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. 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. 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. Many types of inborn errors of BCAA metabolism exist, and are marked by various abnormalities. The most common form is the 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 BCAA and at least one form is correctable by supplementation with 10 mg of biotin daily. BCAA are useful because they are metabolized primarily by muscle. Stress state- e.g surgery, trauma, cirrhosis, infections, fever and starvation--require proportionately more BCAA than other amino acids and probably proportionately more leucine than either valine or isoleucine. BCAA and other amino acids are frequently fed intravenously (TPN) to malnourished surgical patients and in some cases of severe trauma. BCAA, 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. BCAA should also replace the use of steroids as commonly used by weightlifters. Huntington's chorea and anorexic disorders both are characterized by low serum BCAA. These diseases, as well as forms of Parkinson's, may respond to BCAA therapy. BCAA, and particularly leucine, are among the amino acids most essential for muscle health. (http://www.dcnutrition.com).
Structure
Thumb
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
(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
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 KeyInChIKey=ROHFNLRQFUQHCH-YFKPBYRVSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Methyl-branched fatty acid
  • Branched fatty acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Drug
  • Food
Biofunction
  • Essential amino acids
  • Protein component
ApplicationNot Available
Cellular locations
  • Extracellular
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point268 - 288 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility21.5 mg/mLNot Available
LogP-1.52HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility69.8 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.17 m3·mol-1ChemAxon
Polarizability14.16 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0pb9-0900000000-c0176b3cef05fc597576View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0900000000-eb7cee37b9d78694010cView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0900000000-7033b5fdcd4216168462View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-0a4i-0900000000-cd1de48a6db61eb4455eView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-05fr-7900000000-e3b993b282ec2115b484View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9100000000-05b5a7a191a32803595eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0006-9000000000-5302e9c96e75e06e9705View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0006-9000000000-9e70778b46864cd22996View in MoNA
LC-MS/MSLC-MS/MS Spectrum - EI-B (HITACHI M-80) , Positivesplash10-0019-9000000000-6e468213b3429cf627bcView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-4f403c61aaa8a103c049View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-d85fcc558423654f45fdView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-931937d3bdd49b3ae623View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0002-0930000000-aa1256c9224fecacf025View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-0a72425a86f804d3a16bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-4ed8cafebf4ee4e82e6aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-3ac62b780abd90dd28b1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-eb75eb03a58512ea36dfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-001i-0900000000-c0f28e4ebdef67c5b509View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-001i-0900000000-0751a9e803a2e7715d1aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-001i-5900000000-8040fc883917239630a7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-016s-9000000000-a7f9e8d43e66dbd02b6fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-001r-7900000000-da119558c426d9c9d3a1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-000i-9000000000-ae8365105ae2a18e0c10View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-000f-9000000000-228ee614be6648b37433View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-0006-9000000000-d1f3e047af455e156a1eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-0006-9000000000-409dfbdb20719c3afda5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positivesplash10-001i-0900000000-720554d58264a9cfdb67View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-001i-0900000000-90fab591d1f6d6318002View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-001i-0900000000-ed843252a559d532dc5eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
1D NMR13C NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
Biofluid Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Adipose Tissue
  • Adrenal Medulla
  • Bladder
  • Epidermis
  • Fibroblasts
  • Intestine
  • Kidney
  • Muscle
  • Nerve Cells
  • Neuron
  • Placenta
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Testes
Pathways
NameSMPDB LinkKEGG Link
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencySMP00137Not Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencySMP00138Not Available
3-hydroxyisobutyric acid dehydrogenase deficiencySMP00521Not Available
3-hydroxyisobutyric aciduriaSMP00522Not Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type ISMP00237Not Available
3-Methylglutaconic Aciduria Type ISMP00139Not Available
3-Methylglutaconic Aciduria Type IIISMP00140Not Available
3-Methylglutaconic Aciduria Type IVSMP00141Not Available
Amikacin PathwaySMP00253Not Available
Arbekacin Action PathwaySMP00713Not Available
Azithromycin PathwaySMP00247Not Available
Beta-Ketothiolase DeficiencySMP00173Not Available
Chloramphenicol Action PathwaySMP00729Not Available
Clarithromycin PathwaySMP00248Not Available
Clindamycin PathwaySMP00249Not Available
Clomocycline PathwaySMP00262Not Available
Demeclocycline PathwaySMP00290Not Available
Doxycycline PathwaySMP00291Not Available
Erythromycin PathwaySMP00250Not Available
Gentamicin PathwaySMP00254Not Available
Glutaminolysis and CancerSMP02298Not Available
Isobutyryl-coa dehydrogenase deficiencySMP00523Not Available
Isovaleric acidemiaSMP00524Not Available
Isovaleric AciduriaSMP00238Not Available
Josamycin Action PathwaySMP00731Not Available
Kanamycin PathwaySMP00255Not Available
Leucine Stimulation on Insulin SignalingSMP00682Not Available
Lincomycin Action PathwaySMP00728Not Available
Lymecycline PathwaySMP00295Not Available
Maple Syrup Urine DiseaseSMP00199Not Available
Methacycline Action PathwaySMP00727Not Available
Methylmalonate Semialdehyde Dehydrogenase DeficiencySMP00384Not Available
Methylmalonic AciduriaSMP00200Not Available
Minocycline PathwaySMP00292Not Available
Neomycin PathwaySMP00256Not Available
Netilmicin PathwaySMP00257Not Available
Oxytetracycline PathwaySMP00293Not Available
Paromomycin Action PathwaySMP00714Not Available
Propionic AcidemiaSMP00236Not Available
Rolitetracycline Action PathwaySMP00726Not Available
Roxithromycin PathwaySMP00251Not Available
Spectinomycin PathwaySMP00258Not Available
Streptomycin PathwaySMP00259Not Available
Telithromycin PathwaySMP00252Not Available
Tetracycline PathwaySMP00294Not Available
Tigecycline Action PathwaySMP00712Not Available
Tobramycin Action PathwaySMP00711Not Available
Transcription/TranslationSMP00019Not Available
Troleandomycin Action PathwaySMP00730Not Available
Valine, Leucine and Isoleucine DegradationSMP00032map00280
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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 Quantified159 ± 23 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified120 +/- 11 uMAdult (>18 years old)FemaleNormal 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 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 ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableInfant (0-1 year old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedBoth
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified50.773 +/- 61.369 uMNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableInfant (0-1 year old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)FemaleNormal details
FecesDetected and Quantified2014.898 +/- 1413.402 uMNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified2076 +/- 837 nmol/g of fecesAdult (>18 years old)Female
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
SalivaDetected and Quantified6.61 +/- 4.84 uMAdult (>18 years old)Both
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>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 ApplicableAdult (>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 ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified11.3 +/- 7.6 uMAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>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
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 Quantified7.547 +/- 4.945 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified3.5 umol/mmol creatinineAdult (>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
    details
    UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified0.1 (0.0-0.2) 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 Quantified3.0 (1.6-6.0) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified2.8 (1.6-5.4) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    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
      • Mordechai, Hien, ...
    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
    BiofluidStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified74.8 +/- 34.3 uMAdult (>18 years old)BothHeart Transplant details
    BloodDetected and Quantified66.0 +/- 161.0 uMNewborn (0-30 days old)BothPhenylketonuria details
    BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothColorectal cancer details
    BloodDetected and Quantified171 +/- 30 uMAdult (>18 years old)MaleSchizophrenia details
    BloodDetected and Quantified141 +/- 26 uMAdult (>18 years old)FemaleSchizophrenia 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 Quantified11.5 +/- 1.5 uMAdult (>18 years old)BothAlzheimer's disease details
    Cerebrospinal Fluid (CSF)Detected and Quantified16.5 +/- 7.0 uMChildren (1-13 years old)Not Specified
    Leukemia
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified13.1 +/- 5.6 uMChildren (1-13 years old)Not SpecifiedLeukemia details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Irritable bowel syndrome
    details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Ulcerative colitis
    details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothColorectal cancer details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothIrritable Bowel Syndrome details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Crohn’s Disease
    details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Ulcerative Colitis
    details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Colorectal cancer
    details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral cancer details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)FemaleBreast cancer details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedPancreatic cancer details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>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 ApplicableAdult (>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 ApplicableAdult (>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
      • Mordechai, Hien, ...
    details
    UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothColorectal cancer 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
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified5.6052 +/- 3.0948 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    Associated Disorders and Diseases
    Disease References
    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. [17031479 ]
    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. [14992292 ]
    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. [2415198 ]
    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. [16789974 ]
    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. [15911239 ]
    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. [12101068 ]
    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. [12101068 ]
    Associated OMIM IDs
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB001946
    KNApSAcK IDC00001377
    Chemspider ID5880
    KEGG Compound IDC00123
    BioCyc IDLEU
    BiGG ID33942
    Wikipedia LinkLeucine
    NuGOwiki LinkHMDB00687
    Metagene LinkHMDB00687
    METLIN ID24
    PubChem Compound6106
    PDB IDLEU
    ChEBI ID15603
    References
    Synthesis ReferenceLeuchtenberger, Wolfgang; Karrenbauer, Michael; Ploecker, Ulf. Scale-up of an enzyme membrane reactor process for the manufacture of L-enantiomeric compounds. Annals of the New York Academy of Sciences (1984), 434(Enzyme Eng.), 78-86.
    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. [19212411 ]
    2. 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. [6696735 ]
    3. 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. [6198473 ]
    4. 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. [15911239 ]
    5. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [12297216 ]
    6. 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. [14992292 ]
    7. 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. [12101068 ]
    8. Yoshimasa T, Nakao K, Ohtsuki H, Li S, Imura H: Methionine-enkephalin and leucine-enkephalin in human sympathoadrenal system and pheochromocytoma. J Clin Invest. 1982 Mar;69(3):643-50. [7061706 ]
    9. Jansson T, Scholtbach V, Powell TL: Placental transport of leucine and lysine is reduced in intrauterine growth restriction. Pediatr Res. 1998 Oct;44(4):532-7. [9773842 ]
    10. Lichtenstein AH, Hachey DL, Millar JS, Jenner JL, Booth L, Ordovas J, Schaefer EJ: Measurement of human apolipoprotein B-48 and B-100 kinetics in triglyceride-rich lipoproteins using [5,5,5-2H3]leucine. J Lipid Res. 1992 Jun;33(6):907-14. [1512514 ]
    11. Mero A: Leucine supplementation and intensive training. Sports Med. 1999 Jun;27(6):347-58. [10418071 ]
    12. Sakamoto M, Nakao K, Yoshimasa T, Ikeda Y, Suda M, Takasu K, Shimbo S, Yanaihara N, Imura H: Occurrence of methionine-enkephalin-Arg6-Gly7-Leu8 with methionine-enkephalin, leucine-enkephalin and methionine-enkephalin-Arg6-Phe7 in human gastric antrum. J Clin Endocrinol Metab. 1983 Jan;56(1):202-4. [6847871 ]
    13. Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Lazarow A, Nissim I: Brain amino acid requirements and toxicity: the example of leucine. J Nutr. 2005 Jun;135(6 Suppl):1531S-8S. [15930465 ]
    14. Iannoli P, Miller JH, Wang HT, Bode B, Souba WW, Avissar NE, Sax HC: Characterization of L-leucine transport system in brush border membranes from human and rabbit small intestine. Metabolism. 1999 Nov;48(11):1432-6. [10582553 ]

    Enzymes

    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
    L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails
    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
    L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails
    General function:
    Involved in nucleotide binding
    Specific function:
    Not Available
    Gene Name:
    LARS2
    Uniprot ID:
    Q15031
    Molecular weight:
    101975.43
    Reactions
    Adenosine triphosphate + L-Leucine + tRNA(Leu) → Adenosine monophosphate + Pyrophosphate + L-leucyl-tRNA(Leu)details
    Adenosine triphosphate + L-Leucine + tRNA(Leu) → Adenosine monophosphate + Pyrophosphate + L-Leucyl-tRNAdetails
    General function:
    Involved in nucleotide binding
    Specific function:
    Catalyzes the specific attachment of an amino acid to its cognate tRNA in a two step reaction: the amino acid (AA) is first activated by ATP to form AA-AMP and then transferred to the acceptor end of the tRNA. Exhibits a post-transfer editing activity to hydrolyze mischarged tRNAs.
    Gene Name:
    LARS
    Uniprot ID:
    Q9P2J5
    Molecular weight:
    134465.155
    Reactions
    Adenosine triphosphate + L-Leucine + tRNA(Leu) → Adenosine monophosphate + Pyrophosphate + L-leucyl-tRNA(Leu)details
    Adenosine triphosphate + L-Leucine + tRNA(Leu) → Adenosine monophosphate + Pyrophosphate + L-Leucyl-tRNAdetails
    General function:
    Involved in catalytic activity
    Specific function:
    Required for the function of light chain amino-acid transporters. Involved in sodium-independent, high-affinity transport of large neutral amino acids such as phenylalanine, tyrosine, leucine, arginine and tryptophan. Involved in guiding and targeting of LAT1 and LAT2 to the plasma membrane. When associated with SLC7A6 or SLC7A7 acts as an arginine/glutamine exchanger, following an antiport mechanism for amino acid transport, influencing arginine release in exchange for extracellular amino acids. Plays a role in nitric oxide synthesis in human umbilical vein endothelial cells (HUVECs) via transport of L-arginine. Required for normal and neoplastic cell growth. When associated with SLC7A5/LAT1, is also involved in the transport of L-DOPA across the blood-brain barrier, and that of thyroid hormones triiodothyronine (T3) and thyroxine (T4) across the cell membrane in tissues such as placenta. Involved in the uptake of methylmercury (MeHg) when administered as the L-cysteine or D,L-homocysteine complexes, and hence plays a role in metal ion homeostasis and toxicity. When associated with SLC7A5 or SLC7A8, involved in the cellular activity of small molecular weight nitrosothiols, via the stereoselective transport of L- nitrosocysteine (L-CNSO) across the transmembrane. Together with ICAM1, regulates the transport activity LAT2 in polarized intestinal cells, by generating and delivering intracellular signals. When associated with SLC7A5, plays an important role in transporting L-leucine from the circulating blood to the retina across the inner blood-retinal barrier
    Gene Name:
    SLC3A2
    Uniprot ID:
    P08195
    Molecular weight:
    67993.3
    General function:
    Involved in methyltransferase activity
    Specific function:
    Probable S-adenosyl-L-methionine-dependent methyltransferase that acts as a component of the wybutosine biosynthesis pathway. Wybutosine is a hyper modified guanosine with a tricyclic base found at the 3'-position adjacent to the anticodon of eukaryotic phenylalanine tRNA. May methylate the carboxyl group of leucine residues to form alpha- leucine ester residues
    Gene Name:
    LCMT2
    Uniprot ID:
    O60294
    Molecular weight:
    75601.1
    General function:
    Involved in methyltransferase activity
    Specific function:
    Methylates the carboxyl group of the C-terminal leucine residue of protein phosphatase 2A catalytic subunits to form alpha-leucine ester residues.
    Gene Name:
    LCMT1
    Uniprot ID:
    Q9UIC8
    Molecular weight:
    32171.66

    Transporters

    General function:
    Involved in transport
    Specific function:
    Sodium-independent, high-affinity transport of small and large neutral amino acids such as alanine, serine, threonine, cysteine, phenylalanine, tyrosine, leucine, arginine and tryptophan, when associated with SLC3A2/4F2hc. Acts as an amino acid exchanger. Has higher affinity for L-phenylalanine than LAT1 but lower affinity for glutamine and serine. L-alanine is transported at physiological concentrations. Plays a role in basolateral (re)absorption of neutral amino acids. Involved in the uptake of methylmercury (MeHg) when administered as the L-cysteine or D,L-homocysteine complexes, and hence plays a role in metal ion homeostasis and toxicity. Involved in the cellular activity of small molecular weight nitrosothiols, via the stereoselective transport of L-nitrosocysteine (L-CNSO) across the transmembrane. Plays an essential role in the reabsorption of neutral amino acids from the epithelial cells to the bloodstream in the kidney
    Gene Name:
    SLC7A8
    Uniprot ID:
    Q9UHI5
    Molecular weight:
    58381.1