mjl1717
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ornithine -citrilline -argininosuccinate-arginine is the backbone of which cycle?/ which details would you add for Step1
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| meg
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Urea cycle.
I think the fact that first two steps (that is formation of citulline)are in mitochondria and rest in cytoplasm. Citrulline enters cytoplasm.
Any other important?
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| mjl1717
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1)NH4 +HCO3+2ATP+CPSynthtaseI-Carbamoyl Phosphate
2)CP and Ornithine using Ornithine Transcarbamoylase form Citrilline
3)As you said this occurred in the M.Matrix**********************
4)Citrilline crossed into the cytoplasm,now with ASPARTATEand ATP and Arginosuccinate synthetase form Arginosuccinate
5) At this point Fumarase is cleaved off A.Lyase forms Arginine,at this point Urea is cleaved off using Arginase -Ornithine is formed. Ornthine returns from cytoplasm to Mitochondria.
6)It occurs in liver and enters blood for delivery to kidney.
7)Uracil and Orotic acid are increase in Ornithine Transcarbamoylse deficiency and remain the same in Carbamoyl Phosphate deficiency.
8) These conditions are Rx with low protein diet and sodium benzoate or phenylpyruvate to capture excess nitrogen
Id say the entire urea cycle is important for Step1
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| meg
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Thanks 
I think Carbomoyl Phosphatase-2 deficiency for Uracil and Orotic. While it is Carbomoyl Phosphatase- 1 with Urea cycle.
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