1.0 2020-03-18 23:26:07 UTC 2022-12-13 23:36:22 UTC CDB000136 L-Valine Valine, abbreviated as Val or V, one of 20 proteinogenic amino acids, is a non-polar amino acid with the chemical formula HO2CCH(NH2)CH(CH3)2. Valine is an essential amino acid that humans do not synthesize and must acquire from diet, usually from protein sources. Along with leucine and isoleucine, valine is a branched-chain amino acid (BCAA), an amino acid 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. 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 sweet 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 has been established as a useful supplemental therapy for an 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. In sickle-cell disease, the hydrophobic amino acid valine replaces the hydrophilic amino acid glutamic acid in beta-globin, causing it to misfold. The sickle cell hemoglobin is thus prone to aggregation. Hypervalinemia is another example of an inborn error of metabolism involving valine, not the other BCAA. It is caused by a deficiency in the valine transaminase gene (PMID: 6067402). As with most amino acids, L-valine is also found in Cannabis plants (PMID: 6991645). (2S)-2-Amino-3-methylbutanoic acid (S)-Valine 2-Amino-3-methylbutyric acid L-(+)-alpha-Aminoisovaleric acid L-alpha-Amino-beta-methylbutyric acid L-Valin V Val VALINE (2S)-2-Amino-3-methylbutanoate 2-Amino-3-methylbutyrate L-(+)-a-Aminoisovalerate L-(+)-a-Aminoisovaleric acid L-(+)-alpha-Aminoisovalerate L-(+)-Α-aminoisovalerate L-(+)-Α-aminoisovaleric acid L-a-Amino-b-methylbutyrate L-a-Amino-b-methylbutyric acid L-alpha-Amino-beta-methylbutyrate L-Α-amino-β-methylbutyrate L-Α-amino-β-methylbutyric acid (S)-2-Amino-3-methyl-butanoate (S)-2-Amino-3-methyl-butanoic acid (S)-2-Amino-3-methylbutanoate (S)-2-Amino-3-methylbutanoic acid (S)-2-Amino-3-methylbutyrate (S)-2-Amino-3-methylbutyric acid (S)-a-Amino-b-methylbutyrate (S)-a-Amino-b-methylbutyric acid (S)-alpha-Amino-beta-methylbutyrate (S)-alpha-Amino-beta-methylbutyric acid 2-Amino-3-methylbutanoate 2-Amino-3-methylbutanoic acid L Valine C5H11NO2 117.15 117.079 (2S)-2-amino-3-methylbutanoic acid L-valine 72-18-4 CC(C)[C@H](N)C(O)=O InChI=1S/C5H11NO2/c1-3(2)4(6)5(7)8/h3-4H,6H2,1-2H3,(H,7,8)/t4-/m0/s1 KZSNJWFQEVHDMF-BYPYZUCNSA-N belongs to the class of organic compounds known as valine and derivatives. Valine and derivatives are compounds containing valine or a derivative thereof resulting from reaction of valine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. Valine and derivatives Organic compounds Organic acids and derivatives Carboxylic acids and derivatives Amino acids, peptides, and analogues Aliphatic acyclic compounds Amino acids Carbonyl compounds Carboxylic acids Hydrocarbon derivatives L-alpha-amino acids Methyl-branched fatty acids Monoalkylamines Monocarboxylic acids and derivatives Organic oxides Organopnictogen compounds Aliphatic acyclic compound Alpha-amino acid Amine Amino acid Branched fatty acid Carbonyl group Carboxylic acid Fatty acid Fatty acyl Hydrocarbon derivative L-alpha-amino acid Methyl-branched fatty acid Monocarboxylic acid or derivatives Organic nitrogen compound Organic oxide Organic oxygen compound Organonitrogen compound Organooxygen compound Organopnictogen compound Primary aliphatic amine Primary amine Valine or derivatives Common amino acids L-alpha-amino acid proteinogenic amino acid pyruvate family amino acid valine water_solubility 58.5 mg/mL logp -2.26 HANSCH,C ET AL. (1995) melting_point 295 - 300 °C logp -2.29 ALOGPS logs 0.26 ALOGPS logp -2 ChemAxon pka_strongest_acidic 2.72 ChemAxon pka_strongest_basic 9.6 ChemAxon iupac (2S)-2-amino-3-methylbutanoic acid ChemAxon average_mass 117.15 ChemAxon mono_mass 117.079 ChemAxon smiles CC(C)[C@H](N)C(O)=O ChemAxon formula C5H11NO2 ChemAxon inchi InChI=1S/C5H11NO2/c1-3(2)4(6)5(7)8/h3-4H,6H2,1-2H3,(H,7,8)/t4-/m0/s1 ChemAxon inchikey KZSNJWFQEVHDMF-BYPYZUCNSA-N ChemAxon polar_surface_area 63.32 ChemAxon refractivity 29.49 ChemAxon polarizability 12.19 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge 0 ChemAxon formal_charge 0 ChemAxon number_of_rings 0 ChemAxon bioavailability 1 ChemAxon rule_of_five Yes ChemAxon ghose_filter Yes ChemAxon veber_rule Yes ChemAxon mddr_like_rule Yes ChemAxon FDB004905 6050 6287 C00183 16414 DB00161 VAL C00001398 34167 L-valine 5842 Turner CE, Elsohly MA, Boeren EG: Constituents of Cannabis sativa L. XVII. A review of the natural constituents. J Nat Prod. 1980 Mar-Apr;43(2):169-234. doi: 10.1021/np50008a001. 6991645 Dancis J, Hutzler J, Tada K, Wada Y, Morikawa T, Arakawa T: Hypervalinemia. A defect in valine transamination. Pediatrics. 1967 Jun;39(6):813-7. 6067402