Spurs
Forum Newbie
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Hey, I am new here and would like to get a help while I am reading Kaplan notes.
In Urea cycle, two enzymes Carbamoyl phosphate synthesis (CPS) and Ornithine Transcarbamoylase (OTC)
Why No increase in uracil or orotic acid of CPS deficiencies
and Uracil and orotic acid increase in blood and urine of OTC deficiencies.
Thanks!!
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| star1
Forum Guru
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Posts: 826
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For important enzyme you should know the substrate and the product.
in CPS the substrate are NH4, HCO3 and the product are Carbamoyl Phosphate
In OTC the substrate are Carbamoyl Phosphate and Ornithine and the product is Citruline
Now if CPS is deficient then no Carbamoyl will be formed But in case of OTC Carbamoyl will be formed. Got it?
Now if OTC is defficiet the carbamoyl will be accumulated and if this product is not used for urea synthesis because of OTC enzyme defi then it will be shunted toward Purine metabolism and will produced uracil and Orotic acid .
hope this will help
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| Spurs
Forum Newbie
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Awesome, Thanks Star1!
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| mjl1717
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its the fine points!
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