MCCC1, methylcrotonoyl-CoA carboxylase 1

MCCC1, methylcrotonoyl-CoA carboxylase 1

• This gene encodes the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008]

• Gene Synonyms (MCC-B, MCCA, methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, 3-methylcrotonyl-CoA carboxylase 1, 3-methylcrotonyl-CoA carboxylase biotin-containing subunit, 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha, MCCase subunit alpha, methylcrotonoyl-CoA carboxylase 1 (alpha), methylcrotonoyl-CoA carboxylase alpha, methylcrotonoyl-Coenzyme A carboxylase 1 (alpha),)
• NCBI Gene ID: 56922
• Species: Homo sapiens (Human)
• UNIPROT ID#>>A0A0S2Z693
  UNIPROT ID#>>Q68D27
  UNIPROT ID#>>Q96RQ3
• View the NCBI Database for this Gene »

Gene products are often involved in multiple pathways and networks within a living cell. Learn more about other interacting partners.

methylcrotonoyl-CoA carboxylase 1 interacts with:

• 3-oxoacid CoA-transferase 2
  arginyl-tRNA synthetase 2, mitochondrial
  chromosome 15 open reading frame 48
  coiled-coil-helix-coiled-coil-helix domain containing 10
  DDB1 and CUL4 associated factor 15
  hook microtubule tethering protein 3
  matrix AAA peptidase interacting protein 1
  methylcrotonoyl-CoA carboxylase 2
  methyltransferase like 2A
  NADH:ubiquinone oxidoreductase core subunit S7
  neuropeptide B
  pleckstrin homology domain containing A4
  unc-93 homolog B1, TLR signaling regulator
  zinc finger protein 746

Paste a protein or nucleic acid sequence in the box below to confirm that it matches this gene’s reference sequence(s). Click on a link under RELATED ORF CLONES to see how a sequence matches to an experimentally-validated ORF clone.