How would a deficiency in tetrahydrobiopterin production or regeneration lead to symptoms similar to those caused by a deficiency in PAH activity?
=> If there is insufficient tetrahydrobiopterin, then the phenylalanine hydroxylase cannot turn over for renewed catalytic cycles; in effect, the enzyme is inhibited by lack of the properly-reduced cofactor. The consequences are much the same as if the enzyme were simply deficient in activity, that is, elevated levels of phenylalanine and byproducts of its metabolism.
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