Chapter title |
Molecular Pathogenesis of Liver Injury in Hereditary Tyrosinemia 1
|
---|---|
Chapter number | 4 |
Book title |
Hereditary Tyrosinemia
|
Published in |
Advances in experimental medicine and biology, January 2017
|
DOI | 10.1007/978-3-319-55780-9_4 |
Pubmed ID | |
Book ISBNs |
978-3-31-955779-3, 978-3-31-955780-9
|
Authors |
Robert M. Tanguay, Francesca Angileri, Arndt Vogel |
Abstract |
Untreated HT1 rapidly degenerates into very severe liver complications often resulting in liver cancer. The molecular basis of the pathogenic process in HT1 is still unclear. The murine model of FAH-deficiency is a suitable animal model, which represents all phenotypic and biochemical manifestations of the human disease on an accelerated time scale. After removal of the drug 2-(2-N-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC), numerous signaling pathways involved in cell proliferation, differentiation and cancer are rapidly deregulated in FAH deficient mice. Among these, the Endoplasmic reticulum (ER) pathway, the heat stress response (HSR), the Nrf2, MEK and ERK pathways, are highly represented. The p21 and mTOR pathways critical regulators of proliferation and tumorigenesis have also been found to be dysregulated. The changes in these pathways are described and related to the development of liver cancer. |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 15 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 3 | 20% |
Student > Bachelor | 2 | 13% |
Professor | 2 | 13% |
Other | 1 | 7% |
Lecturer | 1 | 7% |
Other | 2 | 13% |
Unknown | 4 | 27% |
Readers by discipline | Count | As % |
---|---|---|
Biochemistry, Genetics and Molecular Biology | 5 | 33% |
Pharmacology, Toxicology and Pharmaceutical Science | 3 | 20% |
Veterinary Science and Veterinary Medicine | 1 | 7% |
Agricultural and Biological Sciences | 1 | 7% |
Immunology and Microbiology | 1 | 7% |
Other | 0 | 0% |
Unknown | 4 | 27% |