LMPD Database

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LMP003171

UniProt Annotations

Entry Information
Gene Namemethylcrotonoyl-CoA carboxylase 2 (beta)
Protein EntryMCCB_HUMAN
UniProt IDQ9HCC0
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9HCC0-1; Sequence=Displayed; Name=2; IsoId=Q9HCC0-2; Sequence=VSP_000069; Note=No experimental confirmation available.;
Biophysicochemical PropertiesKinetic parameters: KM=45 uM for ATP ; KM=74 uM for 3-methylcrotonyl-CoA ; Note=kcat is 4.0 sec(-1).;
Catalytic ActivityATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.
DiseaseMethylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D) [MIM
FunctionCarboxyltransferase 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.
PathwayAmino-acid degradation; L-leucine degradation; (S)-3- hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA
Sequence CautionSequence=AAH14897.1; Type=Frameshift; Positions=359; Evidence= ;
SimilarityBelongs to the AccD/PCCB family.
SimilarityContains 1 carboxyltransferase domain.
Subcellular LocationMitochondrion matrix .
SubunitProbably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits.