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When the amino groups are not recycled for the biosynthesis of new amino acids or other Urea

Urea serves as a main excretory form of amino groups derived from the different amino acids. it is
nitrogenous products, they are funneled into a single excretory end product. synthesized in the liver, transported to the kidneys via the blood, and incorporated in the urine for
excretion. The pathway was elucidated by Hans Krebs and Kurt Henseleit in 1932.
• – organisms that excrete amino nitrogen as ammonia.

• – organisms that excrete amino nitrogen as uric acid →

• – organisms that excrete amino nitrogen as urea.

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The NH4+, coming from the various amino acids, combines with CO2, in the form of HCO3−, to Carbamoyl phosphate combines with ornithine in the mitochondria to form citrulline. This reaction
produce carbamoyl phosphate. This reaction is catalyzed by carbamoyl phosphate synthetase I is catalyzed by ornithine transcarbamoylase.
and occurs inside the liver mitochondria.

Lehninger, Biochemistry, 2005

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Garrett and Grisham, Biochemistry, 2010

Lehninger, Biochemistry, 2005


Citrulline is transported out of the mitochondria and into the cytosol where it reacts with aspartate. Argininosuccinate is cleaved into arginine and fumarate. This reaction is catalyzed by
The product is a compound known as argininosuccinate. This reaction is catalyzed by argininosuccinate lyase (argininosuccinase).
argininosuccinate synthetase.

Lehninger, Biochemistry, 2005

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Arginine is cleaved by the action of arginase, producing urea and ornithine. Ornithine is then The urea cycle is linked to the TCA cycle via fumarate and the transamination of oxaloacetate to
transported across the mitochondrial membrane where it can initiate another round of the cycle. aspartate. The reactions comprising the metabolic links are part of the aspartate-argininosuccinate
shunt.

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