MCCB rabbit pAb

Applications: WB
Reactivity: Human; Mouse;Rat
Source: Rabbit
Dilution: WB 1:500-2000
Immunogen: Synthesized peptide derived from human MCCB AA range: 204-254
Storage_stability: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
Concentration: 1 mg/ml
Observed_band(KD): 60kD
Human_gene_id: 64087
Human_swiss_prot_no: Q9HCC0
Subcellular_location: Mitochondrion matrix .
Other_name: Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)
Background: 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. [provided by RefSeq, Jul 2008],