2-Aminoisobutyric acid

2-Aminoisobutyric acid

2-Aminoisobutyric acid (also known as 2-AIB or alpha-aminoisobutyric acid) is a non-proteinogenic amino acid that is used in the biosynthesis of proteins. It is a white, crystalline solid that is soluble in water and practically insoluble in ethanol and ether.

Structure and properties

2-Aminoisobutyric acid has a chemical formula of C4H9NO2 and a molecular weight of 103.12 g/mol. It is a chiral molecule with two enantiomers, L-2-aminoisobutyric acid and D-2-aminoisobutyric acid. The L-isomer is naturally occurring and is found in certain proteins, while the D-isomer is synthetic.

Biosynthesis

2-Aminoisobutyric acid is synthesized from pyruvate and ammonia in a reaction catalyzed by the enzyme alanine transaminase. This reaction also produces alanine, another amino acid.

Biological role

Although 2-aminoisobutyric acid is not incorporated into proteins, it plays a role in the metabolism of other amino acids. It is a key intermediate in the catabolism of threonine and methionine, two essential amino acids. In addition, it is involved in the biosynthesis of pantothenic acid, a vitamin that is essential for the synthesis of coenzyme A.

Medical significance

Elevated levels of 2-aminoisobutyric acid in the urine, a condition known as hyperaminoaciduria, can be a sign of a rare genetic disorder called 2-aminoisobutyric aciduria. This condition is characterized by a deficiency in the enzyme that breaks down 2-aminoisobutyric acid, leading to its accumulation in the body.

See also

• Non-proteinogenic amino acids
• Amino acid metabolism
• Hyperaminoaciduria