MCCC1 Biotin-attachment 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. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC; Ligase; Mitochondrial
Chromosomal Location of Human Ortholog: 3q27.1
Cellular Component:  3-methylcrotonyl-CoA carboxylase complex, mitochondrial; cytosol; methylcrotonoyl-CoA carboxylase complex; mitochondrial matrix; mitochondrion
Molecular Function:  ATP binding; biotin binding; biotin carboxylase activity; metal ion binding; protein binding
Biological Process:  biotin metabolic process; branched-chain amino acid catabolic process; leucine catabolic process; protein heterooligomerization
Disease: 3-methylcrotonyl-coa Carboxylase 1 Deficiency
Reference #:  Q96RQ3 (UniProtKB)
Alt. Names/Synonyms: 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; DKFZp686B20267; FLJ25545; MCC-B; MCCA; MCCase subunit alpha; MCCC1; methylcrotonoyl-CoA carboxylase 1; methylcrotonoyl-CoA carboxylase 1 (alpha); methylcrotonoyl-CoA carboxylase alpha; Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
Gene Symbols: MCCC1
Molecular weight: 80,473 Da
Basal Isoelectric point: 7.66  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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