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The urea cycle converts highly toxic ammonia to urea for excretion. [1] This cycle was the first metabolic cycle to be discovered by Hans Krebs and Kurt Henseleit in 1932, [2] [3] [4] five years before the discovery of the TCA cycle. The urea cycle was described in more detail later on by Ratner and Cohen.
Ornithine transcarbamylase deficiency also known as OTC deficiency is the most common urea cycle disorder in humans. Ornithine transcarbamylase, the defective enzyme in this disorder, is the final enzyme in the proximal portion of the urea cycle, responsible for converting carbamoyl phosphate and ornithine into citrulline.
L-Ornithine is one of the products of the action of the enzyme arginase on L-arginine, creating urea. Therefore, ornithine is a central part of the urea cycle, which allows for the disposal of excess nitrogen. Ornithine is recycled and, in a manner, is a catalyst. First, ammonia is converted into carbamoyl phosphate (H 2 NC(O)OPO 2−
Mutations in the OTC gene can cause Ornithine Transcarbamylase deficiency. It is classified as a urea cycle disorder due to the fact that without proper OTC function ammonia starts to accumulate in the blood. Accumulation of ammonia in the blood is known as hyperammonemia. Although toxic in excess, ammonia is a nitrogen source for the body.
The enzyme ornithine decarboxylase (EC 4.1.1.17, ODC) catalyzes the decarboxylation of ornithine (a product of the urea cycle) to form putrescine. This reaction is the committed step in polyamine synthesis. [1] In humans, this protein has 461 amino acids and forms a homodimer. [2] In humans, ornithine decarboxylase (ODC) is expressed by the ...
For more information see urea cycle. Pages in category "Urea cycle" The following 21 pages are in this category, out of 21 total.
The excretion of urea is called ureotelism. Land animals, mainly amphibians and mammals, convert ammonia into urea, a process which occurs in the liver and kidney. These animals are called ureotelic. [3] Urea is a less toxic compound than ammonia; two nitrogen atoms are eliminated through it and less water is needed for its excretion.
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