1. Rodent models of pheochromocytoma, parallels in rodent and human tumorigenesis.
- Author
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Lussey-Lepoutre, Charlotte, Buffet, Alexandre, Morin, Aurélie, Goncalves, Judith, and Favier, Judith
- Subjects
PHEOCHROMOCYTOMA ,PARAGANGLIOMA ,GENE expression ,GENETIC mutation ,CARCINOGENESIS - Abstract
Paragangliomas and pheochromocytomas are rare neuroendocrine tumors characterized by a large spectrum of hereditary predisposition. Based on gene expression profiling classification, they can be classically assigned to either a hypoxic/angiogenic cluster (cluster 1 including tumors with mutations in
SDHx ,VHL andFH genes) or a kinase-signaling cluster (cluster 2 consisting in tumors related toRET ,NF1 ,TMEM127 andMAX genes mutations, as well as most of the sporadic tumors). The past 15 years have seen the emergence of an increasing number of genetically engineered and grafted models to investigate tumorigenesis and develop new therapeutic strategies. Among them, only cluster 2-related predisposed models have been successful but grafted models are however available to study cluster 1-related tumors. In this review, we present an overview of existing rodent models targeting predisposition genes involved or not in human pheochromocytoma/paraganglioma susceptibility and their contribution to the improvement of pheochromocytoma experimental research. [ABSTRACT FROM AUTHOR]- Published
- 2018
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