In this study, we investigated whether fumarylacetoacetate hydrolase deficient (FAH<sup>-/-</sup>) pigs, a novel large-animal model of HT1, develop fibrosis and cirrhosis characteristic of the human disease.
Moreover, the highly efficient repopulation of hepatocytes in Fah(-/-) livers prevented the progression of liver fibrosis to cirrhosis and in turn restored liver architecture.
Tyrosinaemia I (fumarylacetoacetate hydrolase deficiency) is an autosomal recessive inborn error of tyrosine metabolism that produces liver failure in infancy or a more chronic course of liver disease with cirrhosis, often complicated by hepatocellular carcinoma, in childhood or early adolescence.