ArticleProceedings of the National Academy of Sciences of the United States of America1998
Hepatocyte injury in tyrosinemia type 1 is induced by fumarylacetoacetate and is inhibited by caspase inhibitors.
Article in Proceedings of the National Academy of Sciences of the United States of America, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 78 citations in OpenAlex.
- Short-term and long-term high-fat diet promote metabolic disorder through reprogramming mRNA mMicrobiome · 2025Article
- Review
- In vivo expansion of gene-targeted hepatocytes through transient inhibition of an essential gene.Science translational medicine · 2025Article
- In vivo quantitative photoacoustic evaluation of the liver and kidney pathology in tyrosinemia.Photoacoustics · 2022Article
- Kidney ECM Pregel Nanoarchitectonics for Microarrays to Accelerate Harvesting Gene-Edited Porcine Primary Monoclonal Spheres.ACS omega · 2022Article
- Toxic Metabolites and Inborn Errors of Amino Acid Metabolism: What One Informs about the Other.Metabolites · 2022Review
- Genome-wide detection of CRISPR editing in vivo using GUIDE-tag.Nature communications · 2022Article
- Fumarylacetoacetate hydrolase gene as a knockout target for hepatic chimerism and donor liver production.Stem cell reports · 2021Review
- Treatment adherence in tyrosinemia type 1 patients.Orphanet journal of rare diseases · 2021Observational
- Review
- Fumarate Mediates a Chronic Proliferative Signal in Fumarate Hydratase-Inactivated Cancer Cells by Increasing Transcription and Translation of Ferritin Genes.Molecular and cellular biology · 2017Article
- Hedgehog signaling pathway regulates liver regeneration in the FahInternational journal of clinical and experimental pathology · 2017Article
- Curative ex vivo liver-directed gene therapy in a pig model of hereditary tyrosinemia type 1.Science translational medicine · 2016Article
- Fumarylacetoacetate hydrolase deficient pigs are a novel large animal model of metabolic liver disease.Stem cell research · 2014Article
- Heat shock response associated with hepatocarcinogenesis in a murine model of hereditary tyrosinemia type I.Cancers · 2014Article
- Clinical and para clinical findings in the children with tyrosinemia referring for liver transplantation.International journal of preventive medicine · 2013Article
- The Caenorhabditis elegans K10C2.4 gene encodes a member of the fumarylacetoacetate hydrolase family: a Caenorhabditis elegans model of type I tyrosinemia.The Journal of biological chemistry · 2008Article
- Article
- Sustained phosphorylation of Bid is a marker for resistance to Fas-induced apoptosis during chronic liver diseases.Gastroenterology · 2006Article
- Current strategies for the treatment of hereditary tyrosinemia type I.Paediatric drugs · 2006Review
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Tyrosinemia type 1, caused by mutations in the fumarylacetoacetate hydrolase gene (Fah), is characterized by severe liver injury. We earlier developed a tyrosinemic mouse model with two genetic defects, Fah and 4-hydroxyphenylpyruvate dioxygenase (Hpd) deficiencies. Apoptosis of hepatocytes was induced and an acute onset of liver failure occurred after administration of homogentisic acid (HGA), the intermediate metabolite between the enzymes HPD and FAH. Cytochrome c was released from mitochondria prior to liver failure in the Fah-/- Hpd-/- double-mutant mice after the administration of HGA. In a cell-free system, the addition of fumarylacetoacetate induced the release of cytochrome c from the mitochondria. We also found that caspase inhibitors were highly effective in preventing the liver failure induced by HGA in the double-mutant mice. Therefore, fumarylacetoacetate apparently induces the release of cytochrome c, which in turn triggers activation of the caspase cascade in hepatocytes of subjects with hereditary tyrosinemia type 1.
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