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Ornithine
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  • Ornithine is an amino acid that plays a role in the urea cycle. Ornithine is abnormally acumulated in the body in ornithine transcarbamylase deficiency.

  • 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 (phosphate-CONH2), which creates one half of urea. Ornithine is converted into a urea derivative at the d (terminal) nitrogen by carbamoyl phosphate.

  • Another nitrogen is added from aspartate, producing the denitrogenated fumarate, and the resulting arginine (a guanidinium compound) is hydrolysed back to ornithine, producing urea. the nitrogens of urea come from the ammonia and aspartate, and the nitrogen in ornithine remains intact.

  • The effects of varying dietary protein intake, and of supplements of ornithine, arginine, lysine, and proline on a patient with hyperornithinemia, hyperammonemia, and homocitrullinuria were investigated in an attempt to understand the condition

  • The homocitrulline excretion is increased with increased dietary lysine, and also by increased protein intake. Supplementary ornithine or arginine lowers plasma ammonia levels and simultaneously decreases the excretion of homocitrulline.

  •  A possible cause of this disease is a defect in the transport of ornithine into the mitochondria. Arginine supplements may be useful in the treatment of the condition.Ornithine increases the production and excretion of Human Growth Hormone (HGH) and is responsible for muscle build-up, decomposing fat and increased energy production.

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