3-Methylcrotonyl-CoA

3-Methylcrotonyl-CoA is a molecule involved in the metabolism of leucine, an essential amino acid. It is a key intermediate in the metabolic pathway known as the leucine degradation pathway. This pathway is responsible for the breakdown of leucine, a process that is crucial for maintaining the balance of nitrogen in the body.

Structure

3-Methylcrotonyl-CoA is a coenzyme A derivative. It consists of a 3-methylcrotonyl group attached to the thiol group of coenzyme A. The 3-methylcrotonyl group is a four-carbon unit with a double bond between the second and third carbons and a methyl group attached to the third carbon.

Role in Metabolism

In the leucine degradation pathway, 3-Methylcrotonyl-CoA is produced from 3-methylglutaconyl-CoA by the enzyme 3-methylcrotonyl-CoA carboxylase. This reaction is a decarboxylation, which means it involves the removal of a carboxyl group (-COOH).

Once formed, 3-Methylcrotonyl-CoA is then converted into 3-hydroxyisobutyryl-CoA by the enzyme 3-hydroxyisobutyryl-CoA hydrolase. This is a hydration reaction, which involves the addition of a water molecule.

Clinical Significance

Deficiencies in the enzyme 3-methylcrotonyl-CoA carboxylase can lead to a condition known as 3-Methylcrotonyl-CoA carboxylase deficiency. This is a rare inherited disorder that prevents the body from properly processing certain proteins. Symptoms can include feeding difficulties, recurrent episodes of vomiting and diarrhea, and lethargy.

See Also

• Leucine
• Metabolism
• 3-Methylcrotonyl-CoA carboxylase deficiency

3-Methylcrotonyl-CoA Resources
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