Carbonic anhydrase 5A, mitochondrial

Carbonic anhydrase 5A, mitochondrial (CA5A) is an enzyme that in humans is encoded by the CA5A gene. This enzyme belongs to the carbonic anhydrase family of enzymes, which catalyze the rapid conversion of carbon dioxide to bicarbonate and protons, a reaction that occurs rather slowly in the absence of a catalyst.

Function

The mitochondrial isozyme of carbonic anhydrase (CA5A) is primarily expressed in the mitochondria of the liver and is involved in ureagenesis and gluconeogenesis, essential processes for the production of urea and glucose. This enzyme may play a significant role in the process of metabolic acidosis.

Structure

The CA5A enzyme is a protein that is encoded by the CA5A gene. The protein is composed of 305 amino acids and has a molecular weight of approximately 34 kDa. The protein has a unique structure, with a central core of 10 alpha helices and a zinc ion coordinated by three histidine residues.

Clinical significance

Mutations in the CA5A gene have been associated with certain medical conditions, such as hyperammonemia, a condition characterized by an excess of ammonia in the blood. Further research is needed to fully understand the role of CA5A in human health and disease.

See also

• Carbonic anhydrase
• Mitochondria
• Metabolic acidosis
• Hyperammonemia