MUT - Methylmalonyl-CoA mutase, mitochondrial - human protein (Function)
 
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MUT »  Methylmalonyl-CoA mutase, mitochondrial   [ EC 5.4.99.2 ]  (MCM)
 
Protein also known as:  Methylmalonyl-CoA isomerase.
Gene name:  MUT
Entry whose protein(s) existence is based on evidence at protein level
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1 53 1
GENE REF ISO

Function

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Overview 
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.  
  • CuratedUniProtKB
GO molecular function 
Cobalamin bindingdefinition[GO:0031419]  
  • IEAUniProtKB KW
Metal ion bindingdefinition[GO:0046872]  
  • IEAUniProtKB KW
Methylmalonyl-CoA mutase activitydefinition[GO:0004494]  
  • IEAEC 2 GO
Modified amino acid bindingdefinition[GO:0072341]  
1
  • IDAUniProtKB
GO biological process 
Homocysteine metabolic processdefinition[GO:0050667]  
1
  • IDAUniProtKB
Post-embryonic developmentdefinition[GO:0009791] silver  
  • IEAOrtholog Compara
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 5.4.99.2: (R)-methylmalonyl-CoA succinyl-CoA.  
  • CuratedUniProtKB
It requires the following cofactor
Adenosylcobalamin.  
  • CuratedUniProtKB
Pathways 
According to KEGG, this protein belongs to the following pathways:
Glyoxylate and dicarboxylate metabolism  hsa00630+4594  
Metabolic pathways  hsa01100+4594  
Propanoate metabolism  hsa00640+4594  
Valine, leucine and isoleucine degradation  hsa00280+4594  
According to Reactome, this protein belongs to the following pathways:
Cobalamin (Cbl, vitamin B12) transport and metabolism  REACT_163862  
Defective MMAA causes methylmalonic aciduria type cblA  REACT_169316  
Defective MUT causes methylmalonic aciduria mut type  REACT_169313  
Propionyl-CoA catabolism  REACT_993  
 
More information is available from:
 

Keywords

Molecular function 
Isomerase  definition   [KW-0413]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P22033
Other
GeneWiki: Methylmalonyl-CoA_mutase
GenomeRNAi: 4594
PRO: PR:P22033