MCCC1 - Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial - human protein (Function)
 
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MCCC1 »  Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial   [ EC 6.4.1.4 ]  (MCCase subunit alpha)
 
Protein also known as:  3-methylcrotonyl-CoA carboxylase biotin-containing subunit.
Gene name:  MCCC1
Entry whose protein(s) existence is based on evidence at protein level
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GENE REF ISO

Function

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Overview 
Biotin-attachment 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.  
1
  • CuratedUniProtKB
GO molecular function 
ATP bindingdefinition[GO:0005524]  
  • IEAUniProtKB KW
Biotin bindingdefinition[GO:0009374]  
1
  • NASUniProtKB
Biotin carboxylase activitydefinition[GO:0004075] silver  
  • IEAInterPro 2 GO
Metal ion bindingdefinition[GO:0046872] silver  
  • IEAInterPro 2 GO
Methylcrotonoyl-CoA carboxylase activitydefinition[GO:0004485]  
2
  • IDAUniProtKB
Protein bindingdefinition[GO:0005515]  
1
  • IPIUniProtKB
GO biological process 
Biotin metabolic processdefinition[GO:0006768]  
1
  • NASUniProtKB
Leucine catabolic processdefinition[GO:0006552]  
1
  • NASUniProtKB
  • IEAUniPathway
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 6.4.1.4: ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) ADP + phosphate + 3-methylglutaconyl-CoA.  
1
  • CuratedUniProtKB
It requires the following cofactor
Biotin.  
  • CuratedUniProtKB
Pathways 
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
Metabolic pathways  hsa01100+56922  
Valine, leucine and isoleucine degradation  hsa00280+56922  
According to Reactome, this protein belongs to the following pathways:
Biotin transport and metabolism  REACT_11153  
Branched-chain amino acid catabolism  REACT_197  
Defective HLCS causes multiple carboxylase deficiency  REACT_169312  
 
More information is available from:
 

Keywords

Molecular function 
Ligase  definition   [KW-0436]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

GenomeRNAi: 56922
PRO: PR:Q96RQ3