Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-04-12 20:31:31 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 IUPAC 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 g/LALOGPS
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 area63.32ChemAxon
rotatable bond count2ChemAxon
refractivity29.49ChemAxon
polarizability12.28ChemAxon
Spectra
Spectra
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
BiofluidStatusValueAgeSexConditionReference
BloodDetected and Quantified212.3 +/- 61.3 uMAdult (>18 years old)Not SpecifiedNormal
BloodDetected and Quantified266.3 +/- 61 uMAdult (>18 years old)Not SpecifiedNormal
BloodDetected and Quantified233.0 (190.0-276.0) uMAdult (>18 years old)BothNormal
BloodDetected and Quantified130.0 +/- 50.0 uMNewborn (0-30 days old)Not SpecifiedNormal
  • Geigy Scient...
BloodDetected and Quantified232.0 +/- 28.0 uMChildren (1-13 year old)MaleNormal
  • Geigy Scient...
BloodDetected and Quantified252.0 +/- 37.0 uMAdult (>18 years old)MaleNormal
  • Geigy Scient...
BloodDetected and Quantified209.0 +/- 31.0 uMAdult (>18 years old)FemaleNormal
  • Geigy Scient...
BloodDetected and Quantified138.00 (86.00-190.0) uMAdult (>18 years old)BothNormal
Cerebrospinal Fluid (CSF)Detected and Quantified20.0 (10.0-30.0) uMAdult (>18 years old)BothNormal
Cerebrospinal Fluid (CSF)Detected and Quantified19 +/- 13 uMNot SpecifiedBothNormal
Cerebrospinal Fluid (CSF)Detected and Quantified15.0 +/- 2.8 uMAdult (>18 years old)BothNormal
  • Geigy Scient...
Cerebrospinal Fluid (CSF)Detected and Quantified23.8 +/- 4.5 uMAdult (>18 years old)Not SpecifiedNormal
Cerebrospinal Fluid (CSF)Detected and Quantified23.9 +/- 6.9 uMAdult (>18 years old)MaleNormal
Cerebrospinal Fluid (CSF)Detected and Quantified14.5 +/- 5.0 uMAdult (>18 years old)FemaleNormal
Cerebrospinal Fluid (CSF)Detected and Quantified18.1 +/- 10.1 uMAdult (>18 years old)Not SpecifiedNormal
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)MaleNormal
UrineDetected and Quantified5.5 (2.7-9.8) umol/mmol creatinineAdult (>18 years old)BothCommentNormal
UrineDetected and Quantified4.93 umol/mmol creatinineAdult (>18 years old)BothNormal
UrineDetected and Quantified12.30 +/- 7.69 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal
UrineDetected and Quantified<5.98-25.62 umol/mmol creatinineAdult (>18 years old)BothNormal
  • David F. Put...
UrineDetected and Quantified2.88 umol/mmol creatinineAdult (>18 years old)MaleNormal
  • Shaykhutdino...
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothCommentNormal
UrineDetected and Quantified0.0177 - 0.0353 umol/mmol creatinineAdult (>18 years old)BothNormal
UrineDetected and Quantified3.15 +/- 1.49 umol/mmol creatinineAdult (>18 years old)BothNormal
UrineDetected and Quantified4.3 (2.4-10.4) umol/mmol creatinineAdult (>18 years old)BothCommentNormal
UrineDetected and Quantified11.58 (8.51 – 15.04) umol/mmol creatinineAdult (>18 years old)BothNormal
  • Lorena Ivona...
UrineDetected and Quantified15.8 (7.4-24.0) umol/mmol creatinineAdult (>18 years old)BothNormal
UrineDetected and Quantified3.4 (2.0-7.7) umol/mmol creatinineAdult (>18 years old)BothCommentNormal
UrineDetected and Quantified0.8 (0.3-1.35) umol/mmol creatinineNewborn (0-30 days old)BothNormal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
UrineDetected and Quantified1.67 +/- 0.80 umol/mmol creatinineChildren (1-13 year old)MaleNormal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
UrineDetected and Quantified3.0 +/- 1.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
UrineDetected and Quantified2.3 +/- 1.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
UrineDetected and Quantified2 umol/mmol creatinineAdult (>18 years old)BothNormal
UrineDetected and Quantified3.355(1.118-5.592) umol/mmol creatinineAdult (>18 years old)BothNormal
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReference
    BloodDetected and Quantified144.2 +/- 61.4 uMAdult (>18 years old)Not SpecifiedHeart Transplant
    BloodDetected and Quantified81.0 +/- 122.0 uMNewborn (0-30 days old)BothPhenylketonuria (PKU)
    BloodDetected and Quantified193.0 (184.0-201.0) uMAdult (>18 years old)BothCommentEpilepsy
    BloodDetected and Quantified202.0 (194.0-209.0) uMAdult (>18 years old)BothCommentEpilepsy
    BloodDetected and Quantified356.0 +/- 541.0 uMNewborn (0-30 days old)BothMaple syrup urine disease (MSUD)
    BloodDetected and Quantified1000.00 (500.00-1500.00) uMChildren (1-13 year old)BothHypervalinemia
    BloodDetected and Quantified58.26 +/- 14.74 uMElderly (>65 years old)BothAlzheimer's disease
    Cerebrospinal Fluid (CSF)Detected and Quantified98.61 uM(N=1) (Cachexia) Not SpecifiedAdult (>18 years old)Not SpecifiedAbnormal
    Cerebrospinal Fluid (CSF)Detected and Quantified21.8 +/- 7.2 uMChildren (1-13 year old)Not SpecifiedCommentLeukemia
    Cerebrospinal Fluid (CSF)Detected and Quantified17.9 +/- 6.7 uMChildren (1-13 year old)Not SpecifiedLeukemia
    Cerebrospinal Fluid (CSF)Detected and Quantified4.13 +/- 1.22 uMAdult (>18 years old)BothAlzheimer's disease
    UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothADPKD
    UrineDetected and Quantified3 (0-14) umol/mmol creatinineAdult (>18 years old)BothLung cancer
    UrineDetected and Quantified15.49 (4.79 – 30.71) umol/mmol creatinineAdult (>18 years old)BothType 1 diabetes Mellitus
    • Lorena Ivona...
    UrineDetected and Quantified0.73 +/- 0.12 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease
    UrineDetected and Quantified118.4 umol/mmol creatinineChildren (1-13 year old)BothHypervalinemia
    UrineDetected and Quantified32.4 umol/mmol creatinineAdult (>18 years old)BothParaquat poisoning
    Associated Disorders and Diseases
    Disease References
    Alzheimer's disease
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    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)Not Available
    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

    Gene Name:
    COMT
    Uniprot ID:
    P21964
    Gene Name:
    BCAT1
    Uniprot ID:
    P54687
    Reactions
    L-Valine + Oxoglutaric acid unknown Alpha-ketoisovaleric acid + L-Glutamic aciddetails
    Gene Name:
    BCAT2
    Uniprot ID:
    O15382
    Reactions
    L-Valine + Oxoglutaric acid unknown Alpha-ketoisovaleric acid + L-Glutamic aciddetails
    Gene Name:
    VARS
    Uniprot ID:
    P26640
    Reactions
    Adenosine triphosphate + L-Valine + tRNA(Val) unknown Adenosine monophosphate + Pyrophosphate + L-valyl-tRNA(Val)details
    Adenosine triphosphate + L-Valine + tRNA(Val) unknown Adenosine monophosphate + Pyrophosphate + L-Valyl-tRNA(Val)details
    Gene Name:
    VARS2
    Uniprot ID:
    Q5ST30
    Reactions
    Adenosine triphosphate + L-Valine + tRNA(Val) unknown Adenosine monophosphate + Pyrophosphate + L-valyl-tRNA(Val)details
    Adenosine triphosphate + L-Valine + tRNA(Val) unknown Adenosine monophosphate + Pyrophosphate + L-Valyl-tRNA(Val)details

    Transporters

    Gene Name:
    SLC16A10
    Uniprot ID:
    Q8TF71