


PCCB Antibody (E-7) is a mouse monoclonal IgG2b 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 is 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 linking fatty acid metabolism to the citric acid cycle. Proper functioning of PCCB is essential for maintaining metabolic homeostasis, and inherited mutations in the PCCB gene 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 (E-7) is an invaluable tool for researchers studying metabolic disorders and the 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 (E-7) | sc-515741 | 200 µg/ml | $322.00 | |||
PCCB Antibody (E-7): m-IgG2b BP-HRP Bundle | sc-548635 | 200 µg Ab; 10 µg BP | $361.00 |