2-Methylbutyryl-CoA

2-Methylbutyryl-CoA is a metabolite involved in the metabolism of amino acids. It is a substrate in the metabolic pathway of isoleucine, an essential amino acid in humans.

Metabolic Pathway[edit | edit source]

2-Methylbutyryl-CoA is produced from 2-Methylbutyryl-CoA dehydrogenase, an enzyme that catalyzes the chemical reaction in the metabolism of isoleucine. This reaction is part of the larger branched-chain amino acid degradation pathway, which is responsible for the breakdown of the essential amino acids leucine, isoleucine, and valine.

The metabolic pathway of isoleucine includes several steps:

Isoleucine is first transaminated to 2-Ketoisocaproate.
2-Ketoisocaproate is then reduced to 2-Methylbutyryl-CoA.
2-Methylbutyryl-CoA is converted to Tiglyl-CoA by the enzyme 2-Methylbutyryl-CoA dehydrogenase.
Tiglyl-CoA is further metabolized to form acetyl-CoA, which enters the citric acid cycle.

Clinical Significance[edit | edit source]

Deficiency in the enzyme 2-Methylbutyryl-CoA dehydrogenase can lead to a buildup of 2-Methylbutyryl-CoA, resulting in 2-Methylbutyrylglycinuria, a rare metabolic disorder. Symptoms of this disorder can include developmental delay, seizures, and hypotonia.