GeneBio Systems
MCCB rabbit pAb
MCCB rabbit pAb
SKU:ES15012
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Size: 100μL
Source:Rabbit
Applications:WB
Reactivity:Human; Mouse;Rat
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],
