PCCA Antibody (F-4): sc-376428

PCCA Antibody (F-4) is a mouse monoclonal IgG2b antibody that detects propionyl Coenzyme A carboxylase alpha polypeptide (PCCA) in mouse, rat, and human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry, and enzyme-linked immunosorbent assay (ELISA). PCCA is a crucial mitochondrial enzyme composed of 703 amino acids, primarily located in the mitochondrial matrix, where PCCA plays a vital role in the catabolism of branched-chain amino acids, odd-chain fatty acids, and other metabolites. PCCA′s location within the mitochondria is essential for energy metabolism and proper functioning of metabolic pathways. PCCA (F-4) antibody recognizes a biotin-dependent enzyme responsible for carboxybiotin formation through ATP hydrolysis, featuring a C-terminal biotin-binding domain and a biotin carboxylase domain. Mutations in the gene encoding PCCA can lead to protein deficiency, resulting in propionic acidemia, a serious autosomal recessive disorder affecting metabolism of methionine, isoleucine, threonine, valine, odd-numbered chain fatty acids, and cholesterol. Two isoforms of PCCA exist due to alternative splicing, which may have implications for functional diversity and regulation in metabolic processes.

The preferred PCCA antibody is PCCA Antibody (D-5): sc-374341.