Methylcrotonyl-CoA carboxylase subunit alpha (MCCC1)

Methylcrotonyl-CoA carboxylase subunit alpha (MCCC1) is the large, biotin-dependent alpha subunit of the mitochondrial enzyme 3-methylcrotonyl-CoA carboxylase. This enzyme forms a heterodimer (with MCCC2) and catalyzes the carboxylation of 3-methylcrotonyl-CoA, a key step in the breakdown of leucine and isovalerate. MCCC1 is required for normal amino acid metabolism and energy production from leucine; mutations in the MCCC1 gene cause methylcrotonylglycinuria, an autosomal recessive metabolic disorder with variable severity. Recent evidence shows MCCC1 can modulate innate immunity by enhancing MAVS-mediated NF-κB activation, increasing type I interferon and pro-inflammatory cytokine expression in response to RNA virus infection. Deficiency or dysfunction of MCCC1 can lead to metabolic acidosis, hypoglycemia, and increased susceptibility to metabolic crises.

Other names

MCCAMCCase subunit alphaMCCCα3-methylcrotonyl-CoA carboxylase 13-methylcrotonyl-CoA carboxylase biotin-containing subunit3-methylcrotonyl-CoA:carbon dioxide ligase subunit alphamethylcrotonoyl-CoA carboxylase alphaMCC-Bmethylcrotonoyl-Coenzyme A carboxylase 1 (alpha)methylcrotonoyl-CoA carboxylase subunit 1

Biotin-dependent carboxylation: MCCC1 catalyzes the ATP-dependent carboxylation of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, critical in leucine/isovalerate metabolism. In immune regulation: Enhancement of MAVS-mediated NF-κB signaling and downstream type I interferons/cytokines in response to viral infection