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Article

Fumarylacetoacetate hydrolase deficient pigs are a novel large animal model of metabolic liver disease.

Stem cell research - 1 Jul 2014

Hickey Raymond D, Mao Shennen A, Glorioso Jaime, Lillegard Joseph B, Fisher James E, Amiot Bruce, Rinaldo Piero, Harding Cary O, Marler Ronald, Finegold Milton J, Grompe Markus, Nyberg Scott L

Abstract excerpt

Hereditary tyrosinemia type I (HT1) is caused by deficiency in fumarylacetoacetate hydrolase (FAH), an enzyme that catalyzes the last step of tyrosine metabolism. The most severe form of the disease presents acutely during infancy, and is characterized by severe liver involvement, most commonly resulting in death if untreated. Generation of FAH(+/-) pigs was previously accomplished by adeno-associated...

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Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

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Identifiers

PMID: 24879068
DOI: 10.1016/j.scr.2014.05.003
PMCID: PMC4096906

Genes

FAH

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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