Animal models of tyrosinemia.

  title={Animal models of tyrosinemia.},
  author={Kimitoshi Nakamura and Yasuhiko Tanaka and Hiroshi Mitsubuchi and Fumio Endo},
  journal={The Journal of nutrition},
  volume={137 6 Suppl 1},
  pages={1556S-1560S; discussion 1573S-1575S}
Hereditary tyrosinemia I (HT I) is a genetic disorder of tyrosine metabolism characterized by progressive liver damage from infancy and by a high risk for hepatocellular carcinoma. HT I is due to mutations in the fumarylacetoacetate hydrolase (Fah) gene, which encodes the last enzyme in the tyrosine catabolic pathway. Disturbances in tyrosine metabolism lead to increased levels of succinylacetone and succinylacetoacetate. However, the mechanisms causing liver failure, cirrhosis, renal tubular… CONTINUE READING


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Hereditary Tyrosinemia

Advances in Experimental Medicine and Biology • 2017
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