LMPD Database

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LMP003840

UniProt Annotations

Entry Information
Gene Namemethylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
Protein EntryMCCA_MOUSE
UniProt IDQ99MR8
SpeciesMouse
Comments
Comment typeDescription
Catalytic ActivityATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.
CofactorName=biotin; Xref=ChEBI:CHEBI:57586; Note=Biotin.;
FunctionBiotin-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. {ECO:0000250}.
PathwayAmino-acid degradation; L-leucine degradation; (S)-3- hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
SimilarityContains 1 ATP-grasp domain. {ECO:0000255|PROSITE- ProRule:PRU00409}.
SimilarityContains 1 biotin carboxylation domain. {ECO:0000305}.
SimilarityContains 1 biotinyl-binding domain. {ECO:0000255|PROSITE-ProRule:PRU01066, ECO:0000305}.
Subcellular LocationMitochondrion matrix {ECO:0000250}.
SubunitProbably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits. {ECO:0000250}.