Tyrosinosis is caused due to deficiency of which enzyme

Tyrosinosis is caused due to deficiency of
which enzyme?
a) Fumarylacetoacetate hydrolase.
b) p-hydroxy phenylpyruvate dehydrogenase.
c) Tyrosine transaminase.
d) Tyrosine ligase.
Correct Answer - A
Answer: A. Fumarylacetoacetate hydrolase
Several metabolic disorders are associated with the tyrosine
catabolic pathway. The probable metabolic defect in type I
tyrosinemia (tyrosinosis) is at fumarylacetoacetate hydrolase.
It is characterized by buildup of too much of the amino acid tyrosine
in the blood and tissues due to an inability to metabolize it
The therapy employs a diet low in tyrosine and phenylalanine.
Untreated acute and chronic tyrosinosis leads to death from liver
failure.