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Human Metabolome Database Version 3.5

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Showing metabocard for L-Valine (HMDB00883)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-04-12 14:31:31 -0600
HMDB ID HMDB00883
Secondary Accession Numbers
  • HMDB34366
Metabolite Identification
Common Name L-Valine
Description Valine (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
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
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 Formula C5H11NO2
Average Molecular Weight 117.1463
Monoisotopic Molecular Weight 117.078978601
IUPAC Name (2S)-2-amino-3-methylbutanoic acid
Traditional IUPAC Name L-valine
CAS Registry Number 72-18-4
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 Key KZSNJWFQEVHDMF-BYPYZUCNSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Amino Acids, Peptides, and Analogues
Class Amino Acids and Derivatives
Sub Class Alpha 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 Parent Alpha Amino Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Food
Biofunction
  • Component of Aminoacyl-tRNA biosynthesis
  • Component of Valine, leucine and isoleucine biosynthesis
  • Essential amino acids
Application Not Available
Cellular locations
  • Extracellular
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 295 - 300 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 58.5 mg/mL Not Available
LogP -2.26 HANSCH,C ET AL. (1995)
Predicted Properties
Property Value Source
Water Solubility 214 g/L ALOGPS
LogP -2.29 ALOGPS
LogP -2 ChemAxon
LogS 0.26 ALOGPS
pKa (strongest acidic) 2.72 ChemAxon
pKa (strongest basic) 9.6 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 2 ChemAxon
Polar Surface Area 63.32 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 29.49 ChemAxon
Polarizability 12.28 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
Gas-MS Spectrum
13C NMR Spectrum
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
[1H,1H] 2D NMR Spectrum
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Fibroblasts
  • Epidermis
Pathways
Name SMPDB Link KEGG Link
Valine, Leucine and Isoleucine Degradation SMP00032 map00280 Link_out
Propanoate Metabolism SMP00016 map00640 Link_out
Transcription/Translation SMP00019 Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
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212.3 +/- 61.3 uM Adult (>18 years old) Not Specified Normal Not Available
Blood Detected and Quantified
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266.3 +/- 61 uM Adult (>18 years old) Not Specified Normal Not Available
Blood Detected and Quantified
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233.0 (190.0-276.0) uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
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130.0 +/- 50.0 uM Newborn (0-30 days old) Not Specified Normal Not Available
Blood Detected and Quantified
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232.0 +/- 28.0 uM Children (1-13 year old) Male Normal Not Available
Blood Detected and Quantified
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252.0 +/- 37.0 uM Adult (>18 years old) Male Normal Not Available
Blood Detected and Quantified
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209.0 +/- 31.0 uM Adult (>18 years old) Female Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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20.0 (10.0-30.0) uM Adult (>18 years old) Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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19 +/- 13 uM Not Specified Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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15.0 +/- 2.8 uM Adult (>18 years old) Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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23.8 +/- 4.5 uM Adult (>18 years old) Not Specified Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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23.9 +/- 6.9 uM Adult (>18 years old) Male Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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14.5 +/- 5.0 uM Adult (>18 years old) Female Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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18.1 +/- 10.1 uM Adult (>18 years old) Not Specified Normal Not Available
Saliva Detected and Quantified
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>10 uM Adult (>18 years old) Both Normal Not Available
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Male Normal Not Available
Urine Detected and Quantified
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5.5 (2.7-9.8) umol/mmol creatinine Adult (>18 years old) Both Normal by GC-MS
Urine Detected and Quantified
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4.93 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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12.30 +/- 7.69 umol/mmol creatinine Adult (>18 years old) Not Specified Normal Not Available
Urine Detected and Quantified
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<5.98-25.62 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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2.88 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Both Normal Urine compound detected by GC-MS
Urine Detected and Quantified
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0.0177 - 0.0353 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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3.15 +/- 1.49 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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4.3 (2.4-10.4) umol/mmol creatinine Adult (>18 years old) Both Normal by LC-MS/MS (Biocrates kit)
Urine Detected and Quantified
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11.58 (8.51 – 15.04) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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3.4 (2.0-7.7) umol/mmol creatinine Adult (>18 years old) Both Normal urine by NMR
Urine Detected and Quantified
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0.8 (0.3-1.35) umol/mmol creatinine Newborn (0-30 days old) Both Normal Not Available
Urine Detected and Quantified
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1.67 +/- 0.80 umol/mmol creatinine Children (1-13 year old) Male Normal Not Available
Urine Detected and Quantified
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3.0 +/- 1.0 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Urine Detected and Quantified
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2.3 +/- 1.2 umol/mmol creatinine Adult (>18 years old) Female Normal Not Available
Urine Detected and Quantified
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2 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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3.355(1.118-5.592) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
144.2 +/- 61.4 uM Adult (>18 years old) Not Specified Heart Transplant Not Available
Blood Detected and Quantified
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81.0 +/- 122.0 uM Newborn (0-30 days old) Both Phenylketonuria (PKU) Not Available
Blood Detected and Quantified
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193.0 (184.0-201.0) uM Adult (>18 years old) Both Epilepsy Acute seizures
Blood Detected and Quantified
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202.0 (194.0-209.0) uM Adult (>18 years old) Both Epilepsy Refractory localization-related epilepsy (RLE)
Blood Detected and Quantified
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356.0 +/- 541.0 uM Newborn (0-30 days old) Both Maple syrup urine disease (MSUD) Not Available
Blood Detected and Quantified
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138.00 (86.00-190.0) uM Adult (>18 years old) Both Hypervalinemia Not Available
Blood Detected and Quantified
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1000.00 (500.00-1500.00) uM Adult (>18 years old) Both Hypervalinemia Not Available
Blood Detected and Quantified
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58.26 +/- 14.74 uM Elderly (>65 years old) Both Alzheimer's disease Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
Article_icon
98.61 uM(N=1) (Cachexia) Not Specified Adult (>18 years old) Not Specified Abnormal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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21.8 +/- 7.2 uM Children (1-13 year old) Not Specified Leukemia Acute Lymphoblastic Leukemia (ALL) with Central...
Cerebrospinal Fluid (CSF) Detected and Quantified
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17.9 +/- 6.7 uM Children (1-13 year old) Not Specified Leukemia Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
Article_icon
4.13 +/- 1.22 uM Adult (>18 years old) Both Alzheimer's disease Not Available
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Both ADPKD Not Available
Urine Detected and Quantified
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3 (0-14) umol/mmol creatinine Adult (>18 years old) Both Lung cancer Not Available
Urine Detected and Quantified
Article_icon
15.49 (4.79 – 30.71) umol/mmol creatinine Adult (>18 years old) Both Type 1 diabetes Mellitus Not Available
Urine Detected and Quantified
Article_icon
0.73 +/- 0.12 umol/mmol creatinine Adult (>18 years old) Both Alzheimer's disease Not Available
Urine Detected and Quantified
Article_icon
15.8 (7.4-24.0) umol/mmol creatinine Adult (>18 years old) Both Hypervalinemia Not Available
Urine Detected and Quantified
Article_icon
118.4 umol/mmol creatinine Adult (>18 years old) Both Hypervalinemia Not Available
Urine Detected and Quantified
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32.4 umol/mmol creatinine Adult (>18 years old) Both Paraquat poisoning Not Available
Associated Disorders and Diseases
Disease References
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 Link_out
    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 Link_out
      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 Link_out
        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 Link_out
          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 Link_out
            Lung Cancer
            • 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 Link_out
              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 Link_out
                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 Link_out
                  Hypervalinemia
                    • http://www.metagene.de/program/d.prg?mp=HYPERVALINEMIA
                    Associated OMIM IDs
                    DrugBank ID DB00161 Link_out
                    Phenol Explorer Compound ID Not Available
                    Phenol Explorer Metabolite ID Not Available
                    FoodDB ID FDB000465
                    KNApSAcK ID C00001398 Link_out
                    Chemspider ID 6050 Link_out
                    KEGG Compound ID C00183 Link_out
                    BioCyc ID VAL Link_out
                    BiGG ID 34167 Link_out
                    Wikipedia Link valine Link_out
                    NuGOwiki Link HMDB00883 Link_out
                    Metagene Link HMDB00883 Link_out
                    METLIN ID 5842 Link_out
                    PubChem Compound 6287 Link_out
                    PDB ID VAL Link_out
                    ChEBI ID 16414 Link_out
                    References
                    Synthesis Reference Kinoshita, Shukuo; Udaka, Shigezo. L-Valine production by fermentation. (1962), 2 pp.
                    Material Safety Data Sheet (MSDS) Download (PDF)
                    General References
                    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 Link_out
                    2. 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 Link_out
                    3. 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 Link_out
                    4. 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 Link_out
                    5. 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 Link_out
                    6. 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 Link_out
                    7. 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 Link_out
                    8. 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 Link_out
                    9. 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 Link_out
                    10. 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 Link_out
                    11. 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 Link_out
                    12. 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 Link_out
                    13. 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 Link_out
                    14. 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 Link_out
                    15. 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 Link_out
                    16. 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 Link_out
                    17. 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 Link_out
                    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 Link_out
                    Enzymes
                    Name: Propionyl-CoA carboxylase beta chain, mitochondrial
                    Reactions:
                    • ATP + propanoyl-CoA + HCO3- = ADP + phosphate + (S)-methylmalonyl-CoA [RN:R01859]
                    Gene Name: PCCB
                    Uniprot ID: P05166 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Catechol O-methyltransferase
                    Reactions:
                    • S-adenosyl-L-methionine + a catechol = S-adenosyl-L-homocysteine + a guaiacol [RN:R07330]
                    Gene Name: COMT
                    Uniprot ID: P21964 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Branched-chain-amino-acid aminotransferase, cytosolic
                    Reactions:
                    • L-leucine + 2-oxoglutarate = 4-methyl-2-oxopentanoate + L-glutamate [RN:R01090]
                    Gene Name: BCAT1
                    Uniprot ID: P54687 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Branched-chain-amino-acid aminotransferase, mitochondrial
                    Reactions:
                    • L-leucine + 2-oxoglutarate = 4-methyl-2-oxopentanoate + L-glutamate [RN:R01090]
                    Gene Name: BCAT2
                    Uniprot ID: O15382 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Valyl-tRNA synthetase
                    Reactions:
                    • ATP + L-valine + tRNAVal = AMP + diphosphate + L-valyl-tRNAVal [RN:R03665]
                    Gene Name: VARS
                    Uniprot ID: P26640 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Valyl-tRNA synthetase, mitochondrial
                    Reactions:
                    • ATP + L-valine + tRNAVal = AMP + diphosphate + L-valyl-tRNAVal [RN:R03665]
                    Gene Name: VARS2
                    Uniprot ID: Q5ST30 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Transporters
                    Name: Monocarboxylate transporter 10
                    Reactions:
                      Gene Name: SLC16A10
                      Uniprot ID: Q8TF71 Link_out
                      Protein Sequence: FASTA
                      Gene Sequence: FASTA