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MCCC1

Official Full Name
methylcrotonyl-CoA carboxylase subunit 1
Organism
Homo sapiens
GeneID
56922
Background
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]
Synonyms
MCCA; MCC-B; MCCCalpha;

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