confused ab OTC deficiency

[Pushtothestars]

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Can't wrap my mind around this-

Is OTC deficiency the same as Carbomyl phosphate def? If not how to differentiate.

How does N-acetylglumate synthase and Oritic Aciduria come to play? I know that N-acetyglumate synthase is the cofactor for CPS I so is it similar too?

 

[Transposony]

Carbamoyl phosphate synthase deficiency will not produce orotic aciduria since Carbamoyl phosphate is not being made (as Carbamoyl phosphate gets converted to Orotic acid & subsequently to Uracil in the cytoplasm)
CPS deficiency is inherited as AR while the OTC deficiency is X-linked recessive.
Since N-acetylglutamate is the cofactor for CPS, it's deficiency will present similar to CPS deficiency.