Methylmalonyl-CoA mutase

MUT
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2XIJ, 2XIQ, 3BIC
Identifiers
Aliases	MUT, MCM, methylmalonyl-CoA mutase, Methylmalonyl Coenzyme-A mutase
External IDs	OMIM: 609058 MGI: 97239 HomoloGene: 20097 GeneCards: MUT
Gene ontology Molecular function • cobalamin binding • modified amino acid binding • isomerase activity • catalytic activity • metal ion binding • intramolecular transferase activity • methylmalonyl-CoA mutase activity Cellular component • mitochondrial matrix • mitochondrion Biological process • short-chain fatty acid catabolic process • cobalamin metabolic process • homocysteine metabolic process • metabolic process • post-embryonic development Sources:Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	4594	17850
Ensembl	ENSG00000146085	ENSMUSG00000023921
UniProt	P22033	P16332
RefSeq (mRNA)	NM_000255	NM_008650
RefSeq (protein)	NP_000246 NP_000246.2	NP_032676.2 NP_032676
Location (UCSC)	Chr 6: 49.43 – 49.46 Mb	Chr 17: 40.93 – 40.96 Mb
PubMed search

methylmalonyl-CoA mutase
Identifiers
EC number	5.4.99.2
CAS number	9023-90-9
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / EGO
Search PMC articles PubMed articles NCBI proteins

Search
PMC	articles
PubMed	articles
NCBI	proteins

2XIJ, 2XIQ, 3BIC

4594

17850

ENSG00000146085

ENSMUSG00000023921

P22033

P16332

NM_000255

NM_008650

NP_000246
NP_000246.2

NP_032676.2
NP_032676

Methylmalonyl-CoA mutase (MCM), mitochondrial, also known as methylmalonyl-CoA isomerase, is a protein that in humans is encoded by the MUT gene. This vitamin B12-dependent enzyme catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA in humans. Mutations in MUT gene may lead to various types of methylmalonic aciduria.

MCM was first identified in rat liver and sheep kidney in 1955. In its latent form, it is 750 amino acids in length. Upon entry to the mitochondria, the 32 amino acid mitochondrial leader sequence at the N-terminus of the protein is cleaved, forming the fully processed monomer. The monomers then associate into homodimers, and bind AdoCbl (one for each monomer active site) to form the final, active holoenzyme form.

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Wikipedia