The enzyme "arginase A" breaks down arginine, an amino acid, into ornithine and urea. Its name is spelled /ɑːrˈdʒɪneɪz ə/ in IPA phonetic transcription. The first syllable is pronounced like "are", followed by a hard "g" sound and then "in". The second syllable is pronounced like the word "ace". The final "a" is pronounced like "uh". The spelling of "arginase A" can be confusing since it is pronounced quite different than it is spelled, but its function is crucial in nitrogen metabolism.
Arginase A is an enzyme that plays a crucial role in metabolism by catalyzing the hydrolysis of arginine to form urea and ornithine. This enzyme is part of the arginine metabolism pathway, which is responsible for the breakdown of arginine, an amino acid that is obtained from the diet or produced by the body.
Arginase A is primarily found in the liver, where it is involved in the urea cycle. This cycle is responsible for the detoxification of ammonia, a byproduct of amino acid metabolism, by converting it into urea, a less toxic compound that can be excreted by the kidneys. The reaction catalyzed by arginase A is the final step of this cycle, and its product ornithine is then recycled to continue the cycle.
In addition to its role in the urea cycle, arginase A also participates in other physiological processes. It is involved in the regulation of nitric oxide (NO) production, as it competes with nitric oxide synthase for the substrate arginine. By decreasing the availability of arginine, arginase A indirectly modulates the production of nitric oxide, a signaling molecule with various physiological functions.
Arginase A is a zinc metalloenzyme, meaning it requires zinc as a cofactor for its enzymatic activity. Deficiency or dysfunction of arginase A can lead to hyperargininemia, a rare genetic disorder characterized by excessive levels of arginine in the blood. This condition can result in symptoms such as intellectual disabilities, developmental delays, liver disease, and elevated ammonia levels.
The word "arginase" is derived from the amino acid "arginine" and the suffix "-ase" which denotes an enzyme. "Arginine" is named after the Greek word "arginos" meaning "bright, shining", as it was first isolated from arginine-rich proteins involved in the repair and regeneration of tissues. The letter "A" in "arginase A" likely represents a specific isoform or variant of the enzyme.