Names & Taxonomy
- Uniprot ID:
- P22033
- Entry Name:
- MUTA_HUMAN
- Status:
- reviewed
- Protein Names:
- Methylmalonyl-CoA mutase, mitochondrial (MCM) (EC 5.4.99.2) (Methylmalonyl-CoA isomerase)
- Gene Names:
- MUT
- Gene Names Primary:
- MUT
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 750
- Sequence:
- MLRAKNQLFLLSPHYLRQVKESSGSRLIQQRLLHQQQPLHPEWAALAKKQLKGKNPEDLIWHTPEGISIKPLYSKRDTMDLPEELPGVKPFTRGPYPTMYTFRPWTIRQYAGFSTVEESNKFYKDNIKAGQQGLSVAFDLATHRGYDSDNPRVRGDVGMAGVAIDTVEDTKILFDGIPLEKMSVSMTMNGAVIPVLANFIVTGEEQGVPKEKLTGTIQNDILKEFMVRNTYIFPPEPSMKIIADIFEYTAKHMPKFNSISISGYHMQEAGADAILELAYTLADGLEYSRTGLQAGLTIDEFAPRLSFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMFQPKNSKSLLLRAHCQTSGWSLTEQDPYNNIVRTAIEAMAAVFGGTQSLHTNSFDEALGLPTVKSARIARNTQIIIQEESGIPKVADPWGGSYMMECLTNDVYDAALKLINEIEEMGGMAKAVAEGIPKLRIEECAARRQARIDSGSEVIVGVNKYQLEKEDAVEVLAIDNTSVRNRQIEKLKKIKSSRDQALAERCLAALTECAASGDGNILALAVDASRARCTVGEITDALKKVFGEHKANDRMVSGAYRQEFGESKEITSAIKRVHKFMEREGRRPRLLVAKMGQDGHDRGAKVIATGFADLGFDVDIGPLFQTPREVAQQAVDADVHAVGISTLAAGHKTLVPELIKELNSLGRPDILVMCGGVIPPQDYEFLFEVGVSNVFGPGTRIPKAAVQVLDDIEKCLEKKQQSV
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix.
Function
- Function:
- Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species.
- Catalytic Activity:
- (R)-methylmalonyl-CoA = succinyl-CoA.
- Cofactor:
- COFACTOR: Name=adenosylcob(III)alamin; Xref=ChEBI:CHEBI:18408;
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=4.7 nM for adenosylcob(III)alamin
- Cross Reference Drug Bank:
- DB00115 DB00200
- Gene Ontology Go:
- mitochondrial matrix
mitochondrion
cobalamin binding
metal ion binding
methylmalonyl-CoA mutase activity
modified amino acid binding
cellular lipid metabolic process
cobalamin metabolic process
fatty acid beta-oxidation
homocysteine metabolic process
post-embryonic development
short-chain fatty acid catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Biological Process:
- cellular lipid metabolic process
cobalamin metabolic process
fatty acid beta-oxidation
homocysteine metabolic process
post-embryonic development
short-chain fatty acid catabolic process
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Molecular Function:
- cobalamin binding
metal ion binding
methylmalonyl-CoA mutase activity
modified amino acid binding - Gene Ontology Cellular Component:
- mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
Acetylation
Cobalamin
Cobalt
Complete proteome
Direct protein sequencing
Disease mutation
Isomerase
Metal-binding
Mitochondrion
Phosphoprotein
Polymorphism
Reference proteome
Transit peptide