Urea Cycle Made Simple - Biochemistry Video

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The Urea cycle is a biochemical pathway in which the free ammonia is converted into urea so that it can be easily excreted.
Urea is the major end product of nitrogen metabolism in humans.
Deficiencies of the various enzymes and transporters involved in the urea cycle can cause urea cycle disorders:

N-Acetylglutamate synthase deficiency
Carbamoyl phosphate synthetase deficiency
Ornithine transcarbamoylase deficiency
Citrullinemia (Deficiency of argininosuccinic acid synthase)
Argininosuccinic aciduria (Deficiency of argininosuccinic acid lyase)
Argininemia (Deficiency of arginase)
Hyperornithinemia, hyperammonemia, homocitrullinuria syndrome (Deficiency of the mitochondrial ornithine transporter)
Most urea cycle disorders are associated with hyperammonemia, however argininemia and some forms of argininosuccinic aciduria do not present with elevated ammonia.

Organisms that cannot easily and quickly remove ammonia usually have to convert it to some other substance, like urea or uric acid, which are much less toxic. Insufficiency of the urea cycle occurs in some genetic disorders (inborn errors of metabolism), and in liver failure. The result of liver failure is accumulation of nitrogenous waste, mainly ammonia, which leads to hepatic encephalopathy.
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Видео Urea Cycle Made Simple - Biochemistry Video канала MEDSimplified