Showing chemical card for (+)-Valine (CFc000333662)
Record Information
Version
1.0
Creation Date
Update Date
2022-09-14 05:13:57 UTC
Chemfont ID
CFc000333662
Molecule Identification
Common Name
(+)-Valine
Definition
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. 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 ).
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.
