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PCCB Antibody (F-6) is a mouse monoclonal IgG1 antibody that detects PCCB in mouse, rat, and human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). PCCB, or Propionyl Coenzyme A carboxylase beta chain, is a crucial 539 amino acid subunit of the propionyl-CoA carboxylase complex, which plays a vital role in the metabolism of propionyl-CoA, an important intermediate in amino acid metabolism and the oxidation of odd-numbered fatty acids. PCCB (F-6) antibody targets a protein primarily located in the mitochondrial matrix, where PCCB catalyzes the biotin-dependent carboxylation of propionyl-CoA to d-methylmalonyl-CoA, a key step in the metabolic pathway that converts propionyl-CoA into succinyl-CoA, ultimately facilitating energy production and glucose synthesis. Proper functioning of PCCB is essential, as inherited mutations in the gene encoding PCCB can lead to propionic acidemia type II, a serious autosomal recessive disorder characterized by a range of symptoms including neutropenia, hypogammaglobulinemia, and developmental delays. Anti-PCCB antibody (F-6) serves as an invaluable tool for researchers studying metabolic disorders and biochemical pathways involving propionyl-CoA, providing insights into the molecular mechanisms underlying these conditions.
Ordering Information
Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
PCCB Antibody (F-6) | sc-515740 | 200 µg/ml | $316.00 | |||
PCCB Antibody (F-6): m-IgG Fc BP-HRP Bundle | sc-541081 | 200 µg Ab; 10 µg BP | $354.00 | |||
PCCB Antibody (F-6): m-IgG1 BP-HRP Bundle | sc-542568 | 200 µg Ab; 20 µg BP | $354.00 |