Predicted to enable fumarylacetoacetase activity. Predicted to be involved in L-phenylalanine catabolic process; homogentisate catabolic process; and tyrosine catabolic process. Is expressed in hypodermis and intestine. Used to study tyrosinemia type I. Human ortholog(s) of this gene implicated in tyrosinemia type I. Orthologous to human FAH (fumarylacetoacetate hydrolase).
WB Description
fah-1 encodes a putative fumarylacetoacetate hydrolase, orthologous to human FAH (OMIM:276700, mutated in type I tyrosinemia), that is required for tyrosine metabolism, resistance to oxidative and protein-folding stresses, and more generally for normally rapid growth, normal locomotion, fertility, and viability; fah-1 is expressed in hypodermis and intestine; the intestines of fah-1(RNAi) animals atrophy rapidly, and show an abnormally active oxidative stress response (seen via gst-4::GFP) and ER stress response (seen via hsp-4::GFP); while RNAi against enzymes upstream of fah-1 suppresses the fah-1(RNAi) phenotype, excess dietary tyrosine or homogentisic acid enhances it, and exogenous succinylacetone (SA) mimics it, consistent with the hypothesis that blocked SA metabolism is toxic in fah-1(RNAi) animals.