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Animal models reveal pathophysiologies of tyrosinemias Endo F; Tanaka Y; Tomoeda K; Tanoue A; Tsujimoto G; Nakamura KJ Nutr 2003[Jun]; 133 (6 Suppl 1): 2063S-2067SThe activity of the enzyme 4-hydroxyphenylpyruvic acid dioxygenase (HPD) is regulated by transcription factors. Mutations in the HPD locus are related to two known distinct diseases: hereditary tyrosinemia type 3 and hawkinsinuria. HPD-deficient mice are a good model with which to examine the biological effects of 4-hydroxyphenylpyruvic acid, which is a keto acid that causes no apparent visceral damage. In contrast, hereditary tyrosinemia type 1, a genetic disease caused by a deficiency of fumarylacetoacetate hydrolase (FAH), induces severe visceral injuries. Mice with FAH deficiency are lethal after birth; thus, efforts to elucidate the mechanisms of the disease process have been impeded. The use of Fah(-/-) Hpd(-/-) double-mutant mice has enabled studies on tyrosinemias, and essential features of visceral injury have been reveale.|*Disease Models, Animal[MESH]|4-Hydroxyphenylpyruvate Dioxygenase/deficiency/genetics[MESH]|Animals[MESH]|Gene Expression/drug effects[MESH]|Homogentisic Acid/pharmacology[MESH]|Hydrolases/deficiency/genetics[MESH]|Liver/metabolism[MESH]|Mice[MESH]|Mice, Knockout[MESH]|Mutation[MESH]|Tyrosinemias/*enzymology[MESH] |
