3-methyl-2-oxobutanoic acid

3-Methyl-2-oxobutanoic acid is a ketone body that is a byproduct of the metabolism of leucine. It is also an intermediate in the isoleucine biosynthesis pathway. 3-Methyl-2-oxobutanoic acid is a 2-oxo monocarboxylic acid and a member of the class of compounds known as methyl-branched fatty acids.

Structure and Properties

3-Methyl-2-oxobutanoic acid is a carboxylic acid with the molecular formula C5H8O3. It has a molecular weight of 116.12 g/mol. The compound is a chiral molecule, meaning it has a non-superimposable mirror image.

Metabolism

3-Methyl-2-oxobutanoic acid is a metabolite of the amino acid leucine. It is produced in the body during the breakdown of leucine, a process that involves several enzymes, including isovaleryl-CoA dehydrogenase and methylmalonyl-CoA mutase.

The compound is also an intermediate in the biosynthesis of isoleucine, another essential amino acid. In this pathway, 3-methyl-2-oxobutanoic acid is converted into 2-oxoisovalerate by the enzyme ketol-acid reductoisomerase.

Biological Significance

As a metabolite of leucine, 3-methyl-2-oxobutanoic acid plays a role in protein synthesis and energy production. It is also involved in the regulation of blood sugar levels and insulin secretion.

In addition, the compound has been studied for its potential role in various diseases. For example, elevated levels of 3-methyl-2-oxobutanoic acid have been associated with maple syrup urine disease, a rare genetic disorder that affects the metabolism of certain amino acids.

See Also

• Leucine
• Isoleucine
• Ketone bodies
• Maple syrup urine disease

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