Methylcrotonyl-CoA
From WikiMD's WELLNESSPEDIA
Methylcrotonyl-CoA
Methylcrotonyl-CoA is an intermediate in the leucine degradation pathway. It is involved in the metabolism of branched-chain amino acids.
Function[edit]
Methylcrotonyl-CoA is converted to 3-methylglutaconyl-CoA by the enzyme methylcrotonyl-CoA carboxylase (MCC). This reaction requires biotin as a cofactor and is an essential step in the catabolism of leucine.
Clinical significance[edit]
Deficiency in methylcrotonyl-CoA carboxylase activity can lead to a metabolic disorder known as 3-Methylcrotonyl-CoA carboxylase deficiency. This condition is characterized by the accumulation of toxic metabolites, which can cause symptoms such as hypotonia, developmental delay, and metabolic acidosis.
Pathway[edit]
The leucine degradation pathway involves several steps:
- Leucine is first transaminated to α-ketoisocaproate.
- α-Ketoisocaproate is then oxidatively decarboxylated to form isovaleryl-CoA.
- Isovaleryl-CoA is converted to methylcrotonyl-CoA.
- Methylcrotonyl-CoA is carboxylated to form 3-methylglutaconyl-CoA.
- Further steps lead to the production of acetyl-CoA and acetoacetate, which enter the citric acid cycle and ketogenesis, respectively.
See also[edit]
References[edit]
External links[edit]
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Methylcrotonyl coenzyme A
