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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-11-03 18:50:56 UTC
HMDB IDHMDB00883
Secondary Accession Numbers
  • HMDB34366
Metabolite Identification
Common NameL-Valine
DescriptionValine (abbreviated as Val or V) is an -amino acid with the chemical formula HO2CCH(NH2)CH(CH3)2. It is named after the plant valerian. L-Valine is one of 20 proteinogenic amino acids. Its codons are GUU, GUC, GUA, and GUG. This essential amino acid is classified as nonpolar. Along with leucine and isoleucine, valine is a branched-chain amino acid. Branched 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 decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)—tyrosine, tryptophan and phenylalanine, as well as methionine—are increased in these conditions. Valine in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. (http://www.dcnutrition.com). In sickle-cell disease, valine substitutes for the hydrophilic amino acid glutamic acid in hemoglobin. Because valine is hydrophobic, the hemoglobin does not fold correctly. Valine is an essential amino acid, hence it must be ingested, usually as a component of proteins.
Structure
Thumb
Synonyms
  1. (2S)-2-Amino-3-methylbutanoate
  2. (2S)-2-Amino-3-methylbutanoic acid
  3. (S)-2-amino-3-methyl-Butanoate
  4. (S)-2-amino-3-methyl-Butanoic acid
  5. (S)-2-Amino-3-methylbutanoate
  6. (S)-2-Amino-3-methylbutanoic acid
  7. (S)-2-Amino-3-methylbutyrate
  8. (S)-2-Amino-3-methylbutyric acid
  9. (S)-a-Amino-b-methylbutyrate
  10. (S)-a-Amino-b-methylbutyric acid
  11. (S)-alpha-Amino-beta-methylbutyrate
  12. (S)-alpha-Amino-beta-methylbutyric acid
  13. (S)-Valine
  14. 2-Amino-3-methylbutanoate
  15. 2-Amino-3-methylbutanoic acid
  16. 2-Amino-3-methylbutyrate
  17. 2-Amino-3-methylbutyric acid
  18. L-(+)-a-Aminoisovalerate
  19. L-(+)-a-Aminoisovaleric acid
  20. L-(+)-alpha-Aminoisovalerate
  21. L-(+)-alpha-Aminoisovaleric acid
  22. L-a-Amino-b-methylbutyrate
  23. L-a-Amino-b-methylbutyric acid
  24. L-alpha-Amino-beta-methylbutyrate
  25. L-alpha-Amino-beta-methylbutyric acid
  26. L-Valine
  27. Valine
Chemical FormulaC5H11NO2
Average Molecular Weight117.1463
Monoisotopic Molecular Weight117.078978601
IUPAC Name(2S)-2-amino-3-methylbutanoic acid
Traditional NameL-valine
CAS Registry Number72-18-4
SMILES
CC(C)[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C5H11NO2/c1-3(2)4(6)5(7)8/h3-4H,6H2,1-2H3,(H,7,8)/t4-/m0/s1
InChI KeyKZSNJWFQEVHDMF-BYPYZUCNSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassAmino Acids, Peptides, and Analogues
ClassAmino Acids and Derivatives
Sub ClassAlpha Amino Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Amino fatty acids(Lipidmaps)
  • alpha-amino acid(ChEBI)
  • branched-chain amino acid(ChEBI)
Substituents
  • Carboxylic Acid
  • Primary Aliphatic Amine (Alkylamine)
Direct ParentAlpha Amino Acids and Derivatives
Ontology
StatusDetected and Quantified
Origin
  • Food
Biofunction
  • Component of Aminoacyl-tRNA biosynthesis
  • Component of Valine, leucine and isoleucine biosynthesis
  • Essential amino acids
ApplicationNot Available
Cellular locations
  • Extracellular
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point295 - 300 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility58.5 mg/mLNot Available
LogP-2.26HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility214.0ALOGPS
logP-2.3ALOGPS
logP-2ChemAxon
logS0.26ALOGPS
pKa (Strongest Acidic)2.72ChemAxon
pKa (Strongest Basic)9.6ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity29.49 m3·mol-1ChemAxon
Polarizability12.28 Å3ChemAxon
Spectra
SpectraGC-MSMS/MSLC-MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Epidermis
  • Fibroblasts
Pathways
NameSMPDB LinkKEGG Link
Propanoate MetabolismSMP00016map00640
Transcription/TranslationSMP00019Not Available
Valine, Leucine and Isoleucine DegradationSMP00032map00280
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified212.3 +/- 61.3 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified266.3 +/- 61 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified233.0 (190.0-276.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified130.0 +/- 50.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified232.0 +/- 28.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified252.0 +/- 37.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified209.0 +/- 31.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified138.00 (86.00-190.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified20.0 (10.0-30.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified19 +/- 13 uMNot SpecifiedBothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified15.0 +/- 2.8 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified23.8 +/- 4.5 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified23.9 +/- 6.9 uMAdult (>18 years old)MaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified14.5 +/- 5.0 uMAdult (>18 years old)FemaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified18.1 +/- 10.1 uMAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal 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 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)BothNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
SalivaDetected and Quantified11.3 +/- 9.4 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified16.2 +/- 12.3 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified6.3 +/- 5.4 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified3.63 +/- 1.66 uMAdult (>18 years old)BothNormal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified47.97 +/- 33.60 uMAdult (>18 years old)BothNormal
    • Dame, ZT. et al. ...
details
SalivaDetected and Quantified10.2 +/- 8 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified8.45 +/- 6.82 uMAdult (>18 years old)FemaleNormal details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)MaleNormal details
UrineDetected and Quantified5.5 (2.7-9.8) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified4.93 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified12.30 +/- 7.69 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified<5.98-25.62 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified2.88 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.0177 - 0.0353 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified3.15 +/- 1.49 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified4.3 (2.4-10.4) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified11.58 (8.51 – 15.04) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Lorena Ivona ŞTEF...
details
UrineDetected and Quantified15.8 (7.4-24.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified3.4 (2.0-7.7) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.8 (0.3-1.35) umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified1.67 +/- 0.80 umol/mmol creatinineChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified3.0 +/- 1.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified2.3 +/- 1.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified2 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified3.355(1.118-5.592) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified144.2 +/- 61.4 uMAdult (>18 years old)Not SpecifiedHeart Transplant details
    BloodDetected and Quantified81.0 +/- 122.0 uMNewborn (0-30 days old)BothPhenylketonuria details
    BloodDetected and Quantified193.0 (184.0-201.0) uMAdult (>18 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified202.0 (194.0-209.0) uMAdult (>18 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified356.0 +/- 541.0 uMNewborn (0-30 days old)BothMaple syrup urine disease (MSUD) details
    BloodDetected and Quantified1000.00 (500.00-1500.00) uMChildren (1-13 years old)BothHypervalinemia details
    BloodDetected and Quantified58.26 +/- 14.74 uMElderly (>65 years old)BothAlzheimer's disease details
    Cerebrospinal Fluid (CSF)Detected and Quantified98.61 uMAdult (>18 years old)Not SpecifiedCachexia details
    Cerebrospinal Fluid (CSF)Detected and Quantified21.8 +/- 7.2 uMChildren (1-13 years old)Not Specified
    Leukemia
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified17.9 +/- 6.7 uMChildren (1-13 years old)Not SpecifiedLeukemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified4.13 +/- 1.22 uMAdult (>18 years old)BothAlzheimer's disease details
    SalivaDetected and Quantified4.93 +/- 2.31 uMAdult (>18 years old)BothOral cavity disorders
      • Sugimoto et al. (...
    details
    SalivaDetected and Quantified4.27 +/- 0.46 uMAdult (>18 years old)MaleAlzheimer's disease details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral squamous cell carcinoma (OSCC) details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral leukoplakia (OLK) details
    SalivaDetected and Quantified4.77 +/- 1.15 uMAdult (>18 years old)MaleFrontotemporal lobe dementia 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 Quantified5.55 +/- 3.92 uMAdult (>18 years old)BothLewy body disease details
    UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothAutosomal dominant polycystic kidney disease (ADPKD) details
    UrineDetected and Quantified3 (0-14) umol/mmol creatinineAdult (>18 years old)BothLung cancer details
    UrineDetected and Quantified15.49 (4.79 – 30.71) umol/mmol creatinineAdult (>18 years old)BothType 1 diabetes Mellitus
      • Lorena Ivona ŞTEF...
    details
    UrineDetected and Quantified0.73 +/- 0.12 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
    UrineDetected and Quantified118.4 umol/mmol creatinineChildren (1-13 years old)BothHypervalinemia details
    UrineDetected and Quantified32.4 umol/mmol creatinineAdult (>18 years old)BothParaquat poisoning 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. Pubmed: 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. Pubmed: 14992292
    Leukemia
    1. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. Pubmed: 15911239
    Lung Cancer
    1. Stretch C, Eastman T, Mandal R, Eisner R, Wishart DS, Mourtzakis M, Prado CM, Damaraju S, Ball RO, Greiner R, Baracos VE: Prediction of skeletal muscle and fat mass in patients with advanced cancer using a metabolomic approach. J Nutr. 2012 Jan;142(1):14-21. doi: 10.3945/jn.111.147751. Epub 2011 Dec 7. Pubmed: 22157537
    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
    Paraquat poisoning
    1. Bairaktari E, Katopodis K, Siamopoulos KC, Tsolas O: Paraquat-induced renal injury studied by 1H nuclear magnetic resonance spectroscopy of urine. Clin Chem. 1998 Jun;44(6 Pt 1):1256-61. Pubmed: 9625050
    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
    Abnormal
    1. Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2008 Oct 25. Pubmed: 18953024
    Hypervalinemia
    1. MetaGene
    Associated OMIM IDs
    DrugBank IDDB00161
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB000465
    KNApSAcK IDC00001398
    Chemspider ID6050
    KEGG Compound IDC00183
    BioCyc IDVAL
    BiGG ID34167
    Wikipedia Linkvaline
    NuGOwiki LinkHMDB00883
    Metagene LinkHMDB00883
    METLIN ID5842
    PubChem Compound6287
    PDB IDVAL
    ChEBI ID16414
    References
    Synthesis ReferenceKinoshita, Shukuo; Udaka, Shigezo. L-Valine production by fermentation. (1962), 2 pp.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. Pubmed: 2026685
    2. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. Pubmed: 12097436
    3. Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. Pubmed: 6696735
    4. Bairaktari E, Katopodis K, Siamopoulos KC, Tsolas O: Paraquat-induced renal injury studied by 1H nuclear magnetic resonance spectroscopy of urine. Clin Chem. 1998 Jun;44(6 Pt 1):1256-61. Pubmed: 9625050
    5. Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. Pubmed: 7729054
    6. Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. Pubmed: 6198473
    7. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. Pubmed: 15911239
    8. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. Pubmed: 12297216
    9. 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
    10. 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
    11. Stickel F, Osterreicher CH, Datz C, Ferenci P, Wolfel M, Norgauer W, Kraus MR, Wrba F, Hellerbrand C, Schuppan D: Prediction of progression to cirrhosis by a glutathione S-transferase P1 polymorphism in subjects with hereditary hemochromatosis. Arch Intern Med. 2005 Sep 12;165(16):1835-40. Pubmed: 16157826
    12. Wudy SA, Hartmann M, Solleder C, Homoki J: Determination of 17alpha-hydroxypregnenolone in human plasma by routine isotope dilution mass spectrometry using benchtop gas chromatography-mass selective detection. Steroids. 2001 Oct;66(10):759-62. Pubmed: 11522338
    13. Szpetnar M, Pasternak K, Boguszewska A: Branched chain amino acids (BCAAs) in heart diseases (ischaemic heart disease and myocardial infarction). Ann Univ Mariae Curie Sklodowska [Med]. 2004;59(2):91-5. Pubmed: 16146056
    14. Hongpaisan J: Inhibition of proliferation of contaminating fibroblasts by D-valine in cultures of smooth muscle cells from human myometrium. Cell Biol Int. 2000;24(1):1-7. Pubmed: 10826768
    15. Deligezer U, Akisik EE, Dalay N: Homozygosity at the C677T of the MTHFR gene is associated with increased breast cancer risk in the Turkish population. In Vivo. 2005 Sep-Oct;19(5):889-93. Pubmed: 16097444
    16. McInturff JE, Wang SJ, Machleidt T, Lin TR, Oren A, Hertz CJ, Krutzik SR, Hart S, Zeh K, Anderson DH, Gallo RL, Modlin RL, Kim J: Granulysin-derived peptides demonstrate antimicrobial and anti-inflammatory effects against Propionibacterium acnes. J Invest Dermatol. 2005 Aug;125(2):256-63. Pubmed: 16098035
    17. Jensen PK, Jacobsen NO: Studies of D-amino acid oxidase activity in human epidermis and cultured human epidermal cells. Arch Dermatol Res. 1984;276(1):57-64. Pubmed: 6142701
    18. Kurpad AV, Regan MM, Raj TD, Gnanou JV, Rao VN, Young VR: The daily valine requirement of healthy adult Indians determined by the 24-h indicator amino acid balance approach. Am J Clin Nutr. 2005 Aug;82(2):373-9. Pubmed: 16087981

    Enzymes

    General function:
    Involved in ligase activity
    Specific function:
    Not Available
    Gene Name:
    PCCB
    Uniprot ID:
    P05166
    Molecular weight:
    58215.13
    References
    1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
    2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
    3. Kim SN, Ryu KH, Lee EH, Kim JS, Hahn SH: Molecular analysis of PCCB gene in Korean patients with propionic acidemia. Mol Genet Metab. 2002 Nov;77(3):209-16. Pubmed: 12409268
    General function:
    Involved in magnesium ion binding
    Specific function:
    Catalyzes the O-methylation, and thereby the inactivation, of catecholamine neurotransmitters and catechol hormones. Also shortens the biological half-lives of certain neuroactive drugs, like L-DOPA, alpha-methyl DOPA and isoproterenol.
    Gene Name:
    COMT
    Uniprot ID:
    P21964
    Molecular weight:
    30036.77
    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-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic aciddetails
    References
    1. Chen CD, Huang TF, Lin CH, Guan HH, Hsieh YC, Lin YH, Huang YC, Liu MY, Chang WC, Chen CJ: Purification, crystallization and preliminary X-ray crystallographic analysis of branched-chain aminotransferase from Deinococcus radiodurans. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2007 Jun 1;63(Pt 6):492-4. Epub 2007 May 5. Pubmed: 17554170
    2. Beck HC: Branched-chain fatty acid biosynthesis in a branched-chain amino acid aminotransferase mutant of Staphylococcus carnosus. FEMS Microbiol Lett. 2005 Feb 1;243(1):37-44. Pubmed: 15667998
    3. Saito M, Nishimura K, Wakabayashi S, Kurihara T, Nagata Y: Purification of branched-chain amino acid aminotransferase from Helicobacter pylori NCTC 11637. Amino Acids. 2007 Sep;33(3):445-9. Epub 2006 Nov 2. Pubmed: 17077963
    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-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic aciddetails
    General function:
    Involved in nucleotide binding
    Specific function:
    Not Available
    Gene Name:
    VARS
    Uniprot ID:
    P26640
    Molecular weight:
    140474.755
    Reactions
    Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-valyl-tRNA(Val)details
    Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-Valyl-tRNA(Val)details
    References
    1. Zhu B, Zhao MW, Eriani G, Wang ED: A present-day aminoacyl-tRNA synthetase with ancestral editing properties. RNA. 2007 Jan;13(1):15-21. Epub 2006 Nov 9. Pubmed: 17095543
    2. Laforest MJ, Delage L, Marechal-Drouard L: The T-domain of cytosolic tRNAVal, an essential determinant for mitochondrial import. FEBS Lett. 2005 Feb 14;579(5):1072-8. Pubmed: 15710393
    General function:
    Involved in nucleotide binding
    Specific function:
    Not Available
    Gene Name:
    VARS2
    Uniprot ID:
    Q5ST30
    Molecular weight:
    102414.65
    Reactions
    Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-valyl-tRNA(Val)details
    Adenosine triphosphate + L-Valine + tRNA(Val) → Adenosine monophosphate + Pyrophosphate + L-Valyl-tRNA(Val)details

    Transporters

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