Carbonic anhydrases (CAs), also designated carbonate dehydratases or carbonate hydrolyases, form a large family of genes that encode zinc metalloenzymes of great physiologic importance. As catalysts of the reversible hydration of carbon dioxide, these enzymes participate in a variety of biologic processes, including respiration, acid-base balance, bone resorption and calcification as well as the formation of aqueous humor, cerebrospinal fluid, saliva and gastric acid. Genes in the ?carbonic anhydrase family encode either active carbonic anhydrase isozymes or catalytic?(devoid of CO2 hydration activity) carbonic anhydrase-related proteins. Human CA I (CA1) is encoded by the CA1 gene, which maps to a region on chromosome 8 that harbors a cluster of CA genes. CA I localizes to the cytoplasm and research indicates that a severe deficiency of CA I does not result in any obvious hematological or renal consequences.