Protein: 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) (Q3ULD5)
Organism: Mus musculus (Mouse)
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.
Subcellular location: Mitochondrion matrix {ECO:0000250}.
Sequence: MWGALRSALRPCCRAAVPPQRAYHGDSVARLGTQPDSASSTYQENYEQMKALVSQLHERAQYVRLGGSEKARARHTSRGKLLPRDRIDNLIDPGSPFLEFSQFAGYQLYGDEEVPAGGIITGIGRVSGVECMIVANDATVKGGTYYPVTVKKHVRAQEIALQNRLPCIYLVDSGGANLPRQADTFPDRDHFGRIFYNQAIMSSKNITQIAVVMGSCTAGGAYVPAMADENIIVQKQGTIFLAGPPLVKAATGEEVSAEDLGGADLHCRKSGVTDHYALDDHHALHLTRKVVRSLNYQKKMDVTIEPSEEPLFPADELYGIVGANLKRSFDVREVIARIVDGSRFNEFKALYGDTLVTGFARIFGYPVGIIGNNGVLFSESAKKGAHFVQLCCQRNIPLLFLQNITGFMVGRDYEAEGIAKDGAKMVAAVACAKVPKITVIIGGSYGAGNYGMCGRAYSPRFLYMWPNARISVMGGEQAATVLATVARDQKAREGKQFSSAEEAALKEPIIKRFEEEGNPYYSSARLWDDGIIDPVDTRLVLGLSLSAALNAPIQRTDFGIFRM
InterPro domains: - IPR045190: Methylcrotonoyl-CoA carboxylase beta chain MCCB/AccD1-like (family) [24-563]
- IPR011762: Acetyl-coenzyme A carboxyltransferase, N-terminal (domain) [49-306]
- IPR029045: ClpP/crotonase-like domain superfamily (homologous_superfamily) [50-563]
- IPR034733: Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta (domain) [75-554]
- IPR011763: Acetyl-coenzyme A carboxyltransferase, C-terminal (domain) [309-555]
Interaction partners: - Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
- Propionyl-CoA carboxylase alpha chain, mitochondrial
- Isovaleryl-CoA dehydrogenase, mitochondrial
- Enoyl-CoA hydratase, mitochondrial
- Trifunctional enzyme subunit alpha, mitochondrial
- Peroxisomal bifunctional enzyme
- Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
- Methylglutaconyl-CoA hydratase, mitochondrial
- Biotin--protein ligase
- Pyruvate carboxylase, mitochondrial
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0016874 (ligase activity), GO:0016885 (ligase activity, forming carbon-carbon bonds), GO:0016421 (CoA carboxylase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044281 (small molecule metabolic process), GO:0009056 (catabolic process), GO:0044238 (primary metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0006520 (amino acid metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:0044282 (small molecule catabolic process), GO:1901575 (organic substance catabolic process), GO:0006082 (organic acid metabolic process), GO:0044248 (cellular catabolic process), GO:0009081 (branched-chain amino acid metabolic process), GO:0016054 (organic acid catabolic process), GO:1901565 (organonitrogen compound catabolic process), GO:0043436 (oxoacid metabolic process), GO:1901605 (alpha-amino acid metabolic process), GO:0009063 (amino acid catabolic process), GO:0006551 (leucine metabolic process), GO:0019752 (carboxylic acid metabolic process), GO:1901606 (alpha-amino acid catabolic process), GO:0046395 (carboxylic acid catabolic process), GO:0009083 (branched-chain amino acid catabolic process), GO:0006552 (leucine catabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)