Names & Taxonomy
- Uniprot ID:
- Q9HCC0
- Entry Name:
- MCCB_HUMAN
- Status:
- reviewed
- Protein Names:
- Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)
- Gene Names:
- MCCC2 MCCB
- Gene Names Primary:
- MCCC2
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 563
- Sequence:
- MWAVLRLALRPCARASPAGPRAYHGDSVASLGTQPDLGSALYQENYKQMKALVNQLHERVEHIKLGGGEKARALHISRGKLLPRERIDNLIDPGSPFLELSQFAGYQLYDNEEVPGGGIITGIGRVSGVECMIIANDATVKGGAYYPVTVKKQLRAQEIAMQNRLPCIYLVDSGGAYLPRQADVFPDRDHFGRTFYNQAIMSSKNIAQIAVVMGSCTAGGAYVPAMADENIIVRKQGTIFLAGPPLVKAATGEEVSAEDLGGADLHCRKSGVSDHWALDDHHALHLTRKVVRNLNYQKKLDVTIEPSEEPLFPADELYGIVGANLKRSFDVREVIARIVDGSRFTEFKAFYGDTLVTGFARIFGYPVGIVGNNGVLFSESAKKGTHFVQLCCQRNIPLLFLQNITGFMVGREYEAEGIAKDGAKMVAAVACAQVPKITLIIGGSYGAGNYGMCGRAYSPRFLYIWPNARISVMGGEQAANVLATITKDQRAREGKQFSSADEAALKEPIIKKFEEEGNPYYSSARVWDDGIIDPADTRLVLGLSFSAALNAPIEKTDFGIFRM
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix
Function
- Function:
- 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.
- Pathway:
- Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
- Catalytic Activity:
- ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: ; Kinetic parameters: KM=45 uM for ATP
- Cross Reference Drug Bank:
- DB00121
- Gene Ontology Go:
- cytosol
mitochondrial matrix
mitochondrion
ATP binding
methylcrotonoyl-CoA carboxylase activity
biotin metabolic process
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
coenzyme A metabolic process
leucine catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Biological Process:
- biotin metabolic process
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
coenzyme A metabolic process
leucine catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Molecular Function:
- ATP binding
methylcrotonoyl-CoA carboxylase activity - Gene Ontology Cellular Component:
- cytosol
mitochondrial matrix
mitochondrion - Keywords:
- ATP-binding
Acetylation
Alternative splicing
Complete proteome
Direct protein sequencing
Disease mutation
Ligase
Mitochondrion
Nucleotide-binding
Reference proteome
Transit peptide