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Human 3-methylcrotonyl-CoA carboxylase in BCCP-H1 state with MCoA
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Structural highlights
DiseaseMCCB_HUMAN 3-methylcrotonyl-CoA carboxylase deficiency. The disease is caused by variants affecting the gene represented in this entry. FunctionMCCB_HUMAN Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.[1] References
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This page was last modified 07:15, 3 July 2024.