How Do You Spell ORNITHINE CARBAMOYLTRANSFERASE?

1. Ornithine carbamoyltransferase (OTC) is an enzyme that plays a crucial role in the urea cycle, a metabolic pathway responsible for the elimination of excess nitrogen from the body. Specifically, OTC facilitates the conversion of ornithine, an amino acid, to citrulline, another amino acid. This process occurs in the mitochondria of liver cells, where OTC is primarily found.

   The conversion of ornithine to citrulline involves the transfer of a carbamoyl group from carbamoyl phosphate to ornithine. This reaction is catalyzed by OTC, ensuring the continuous flow of the urea cycle and the subsequent removal of toxic ammonia from the body. The enzymatic activity of OTC is tightly regulated and controlled by various factors, including the concentration of substrates and products, as well as the presence of other enzymes and co-factors.

   Deficiencies in OTC activity lead to a genetic disorder called ornithine transcarbamylase deficiency (OTC deficiency) which is characterized by the buildup of ammonia and toxic metabolites in the blood. This condition can result in severe neurological symptoms, liver damage, and even death if left untreated. OTC deficiency is an X-linked disorder, primarily affecting males, and often presents in the early days or months of life.

   In summary, ornithine carbamoyltransferase is an essential enzyme involved in the urea cycle, responsible for the conversion of ornithine to citrulline. Its activity is crucial for the removal of nitrogen from the body and the prevention of ammonia toxicity.

Common Misspellings for ORNITHINE CARBAMOYLTRANSFERASE

The word "ornithine" is derived from the Latin word "ornis", meaning "bird", as ornithine was first discovered in the urine of birds.

"Carbamoyltransferase" is derived from "carbamoyl", which is a combination of "carbamic acid" and "amide". "Carbamic acid" originates from "carbamide", the older name for urea, and "amide" refers to the functional group -CONH2.

Hence, "ornithine carbamoyltransferase" refers to an enzyme that transfers a carbamoyl group from carbamoyl phosphate to ornithine, originally identified in birds.