Article
From Nf1 to Sdhb knockout: Successes and failures in the quest for animal models of pheochromocytoma.
Molecular and cellular endocrinology - 5 Feb 2016
Lepoutre-Lussey Charlotte, Thibault Constance, Buffet Alexandre, Morin Aurélie, Badoual Cécile, Bénit Paule, Rustin Pierre, Ottolenghi Chris, Janin Maxime, Castro-Vega Luis-Jaime, Trapman Jan, Gimenez-Roqueplo Anne-Paule, Favier Judith
Abstract excerpt
Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors characterized by a high frequency of hereditary forms. Based on transcriptome classification, PPGL can be classified in two different clusters. Cluster 1 tumors are caused by mutations in SDHx, VHL and FH genes and are characterized by a pseudohypoxic signature. Cluster 2 PPGL carry mutations in RET, NF1, MAX or TMEM127 genes and display...
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