HHH syndrome is due to deficiency of which enzyme?: (A) Arginase, (B) Arginosuccinase, (C) Ornithine transcarbomylase, (D) None
Answer is C. Ans. is. c' i.e., Ornithine transcarbomylase Actually there is no molecular deficiency of any enzyme but functional deficiency.In HHH syndrome there is deficiency/defect in Ornithine transpoer across the mitochondrial membrane which causes functional deficiency of :?Ornithine transcarbomylaseOrnithine -8- aminotrans ferase (OAT)