MCCC2 Products

Description

MCCC2 encodes the small 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.

Bioinformatics

Entrez	64087 Human
Uniprot	NM_022132 Human NP_071415 Human NP_071415.1 Human Q9HCC0 Human
Product By Gene ID	64087
Alternate Names	3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta MCCase subunit beta MCCBEC 6.4.1.4 methylcrotonoyl-CoA carboxylase 2 (beta) methylcrotonoyl-Coenzyme A carboxylase 2 (beta) mitochondrial non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase