Anti-Human CA5A Antibody Pair

Product Description: Carbonic anhydrase 5A (CA5A) is a mitochondrial enzyme that plays a crucial role in maintaining acid-base balance by catalyzing the reversible hydration of carbon dioxide to bicarbonate. This reaction is vital for various metabolic processes, particularly in the liver, where CA5A supports other enzymes involved in energy production and the urea cycle, which detoxifies ammonia. The enzyme's properties include a high catalytic efficiency and a unique active site structure compared to other carbonic anhydrases, involving cysteine residues instead of histidine. Deficiencies in CA5A due to mutations in the CA5A gene can lead to carbonic anhydrase VA deficiency, a rare inherited disorder characterized by severe metabolic crises, including poor feeding, rapid breathing, and seizures, particularly in infancy. Protein Function: Mitochondrial carbonic anhydrase that catalyzes the reversible conversion of carbon dioxide to bicarbonate/HCO3 (PubMed:24530203, PubMed:8356065). Mitochondria are impermeable to HCO3, and thus this intramitochondrial carbonic anhydrase is pivotal in providing HCO3 for multiple mitochondrial enzymes that catalyze the formation of essential metabolites of intermediary metabolism in the urea and Krebs cycles (PubMed:24530203) [The Uniprot Consortium]