Question

If someone has Argininosuccinate Synthetase deficiency, then why do they avoid certain protein rich food (steak)...

If someone has Argininosuccinate Synthetase deficiency, then why do they avoid certain protein rich food (steak) but crave specific protein-rich food (like Peanut butter) and nonedible things like paper/cardboard.

Homework Answers

Answer #1

Argininosuccinate Synthetase enzyme play a critical role in urea cycle also called "Ornithine" cycle. Urea cycle constitutes multiple of steps to convert highly toxic compounds Ammonia (NH3) into less toxic and excretable form 'urea'. This cycle operates in liver. Argininosuccinate synthetase convert citrulline into arginine and urea. Protein metabolism generates high amount of ammonia and if there is deficiency in this enzyme then the accumulation of ammonia takes place in the body which is highly toxic. So protein rich diets should be avoided.

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