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MCCC1 (NM_020166) - methylcrotonoyl-CoA carboxylase 1

Methylcrotonoyl-Coa Carboxylase Subunit Alpha, Mitochondrial Isoform 1 Precursor

PTM Interaction Network Visualisation

Protein summary

This is preferred isoform of MCCC1 protein. View all 2 isoforms
MCCC1: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 precursor
Description:

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].

Strand
-
Chromosome
3
Protein
725 residues
All mutations
195
PTM sites
18
CDS
182,733,225 - 182,817,228
Transcription
182,733,005 - 182,817,375
10.62% of sequence is predicted to be disordered

Usage summary

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External references

Mappings retrieved from NCBI & UniProt.
RefSeq
Entrez
gene: 56922
UniProt
Ensembl