Tags: Disease.
Hawkinsinuria also called 4-Alpha-hydroxyphenylpyruvate hydroxylase deficiency is an autosomal dominant metabolic disorder affecting the metabolism of tyrosine. Normally the breakdown of the amino acid tyrosine involves the conversion of 4-hydroxyphenylpyruvate to homogentisate by 4-Hydroxyphenylpyruvate dioxygenase. Complete deficiency of this enzyme would lead to tyrosinemia III.