Isovaleryl-CoA dehydrogenase: Difference between revisions
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{{DISPLAYTITLE:Isovaleryl-CoA dehydrogenase}} | {{DISPLAYTITLE:Isovaleryl-CoA dehydrogenase}} | ||
'''Isovaleryl-CoA dehydrogenase''' is an enzyme that plays a crucial role in the metabolism of [[branched-chain amino acids]], specifically [[leucine]]. It is a member of the [[acyl-CoA dehydrogenase]] family, which is involved in the [[beta-oxidation]] of fatty acids. | '''Isovaleryl-CoA dehydrogenase''' is an enzyme that plays a crucial role in the metabolism of [[branched-chain amino acids]], specifically [[leucine]]. It is a member of the [[acyl-CoA dehydrogenase]] family, which is involved in the [[beta-oxidation]] of fatty acids. | ||
Revision as of 04:17, 16 February 2025
Isovaleryl-CoA dehydrogenase is an enzyme that plays a crucial role in the metabolism of branched-chain amino acids, specifically leucine. It is a member of the acyl-CoA dehydrogenase family, which is involved in the beta-oxidation of fatty acids.
Function
Isovaleryl-CoA dehydrogenase catalyzes the third step in the breakdown of leucine, converting isovaleryl-CoA to 3-methylcrotonyl-CoA. This reaction involves the removal of electrons from isovaleryl-CoA, which are transferred to the electron transfer flavoprotein (ETF), ultimately contributing to the electron transport chain and ATP production.
Structure
The enzyme is a homotetramer, meaning it consists of four identical subunits. Each subunit contains a flavin adenine dinucleotide (FAD) cofactor, which is essential for its dehydrogenase activity. The structure of isovaleryl-CoA dehydrogenase has been elucidated through X-ray crystallography, revealing details about its active site and substrate binding.
Clinical significance
Deficiency in isovaleryl-CoA dehydrogenase activity leads to a metabolic disorder known as isovaleric acidemia. This condition is characterized by the accumulation of isovaleric acid, which can cause severe metabolic acidosis, a distinctive "sweaty feet" odor, and neurological complications. Early diagnosis and management are crucial to prevent serious outcomes.
Related enzymes
Isovaleryl-CoA dehydrogenase is part of a larger family of acyl-CoA dehydrogenases, which includes:
- Medium-chain acyl-CoA dehydrogenase (MCAD)
- Short-chain acyl-CoA dehydrogenase (SCAD)
- Very long-chain acyl-CoA dehydrogenase (VLCAD)
