PCCA Antibody (H-1): sc-393527

PCCA Antibody (H-1) is a mouse monoclonal IgG1 antibody that detects propionyl Coenzyme A carboxylase alpha polypeptide (PCCA) in mouse, rat, and human samples through western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), 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 in the mitochondria is essential for energy metabolism and proper functioning of metabolic pathways. This biotin-dependent enzyme forms carboxybiotin through ATP hydrolysis, featuring a C-terminal biotin-binding domain and a biotin carboxylase domain. Mutations in the gene encoding PCCA can lead to 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 influence functional diversity and regulation. anti-PCCA antibody (H-1) serves as an invaluable tool for researchers studying metabolic disorders and mitochondrial function.

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