The inherited disorder phenylketonuria (PKU) is caused by a defect in the metabolism of an amino acid, leading to its accumulation and a variety of neurological problems if not treated. This disorder is the result of a deficiency in which of the following enzymes?

1
Phenylalanine hydroxylase
2
Tyrosinase
3
Glucose-6-phosphate dehydrogenase
4
Uridine diphosphate glucuronosyltransferase

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