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Article

Chronic Phenotype Characterization of a Large-Animal Model of Hereditary Tyrosinemia Type 1.

The American journal of pathology - 1 Jan 2017

Elgilani Faysal, Mao Shennen A, Glorioso Jaime M, Yin Meng, Iankov Ianko D, Singh Anisha, Amiot Bruce, Rinaldo Piero, Marler Ronald J, Ehman Richard L, Grompe Markus, Lillegard Joseph B, Hickey Raymond D, Nyberg Scott L

Abstract excerpt

Hereditary tyrosinemia type 1 (HT1) is an autosomal recessive disease caused by deficiency in fumarylacetoacetate hydrolase, the last enzyme in the tyrosine catabolic pathway. In this study, we investigated whether fumarylacetoacetate hydrolase deficient (FAH-/-) pigs, a novel large-animal model of HT1, develop fibrosis and cirrhosis characteristic of the human disease. FAH-/- pigs were treated with the...

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Identifiers

PMID: 27855279
DOI: 10.1016/j.ajpath.2016.09.013
PMCID: PMC5225308

Genes

FAH

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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