This gene 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.
Categories
Primary Antibodies
Clonality
polyclonal
Description
Carboxyltransferase 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.
Host
Rabbit
Immunogen
methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
Isotype
IgG
Molecular Weight
61 kDa
Reactivity
Human, Mouse, Rat
Regulatory
RUO
Synonyms
MCCB
Uniprot
Q9HCC0
Gene Id
64087
Research Area
Metabolism
Form
liquid
Format
liquid
Purification
Immunogen affinity purified
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)