Total loss of VHL gene function impairs neuroendocrine cancer cell fitness due to excessive HIF2α activity

Abstract excerpt

Loss-of-function germline von Hippel–Lindau ( VHL ) tumor suppressor mutations cause VHL disease, which predisposes individuals to kidney cancer, hemangioblastomas, and paragangliomas. The risk that a given VHL disease family will manifest some or all these tumor types is profoundly influenced by the VHL allele it carries. For example, almost all VHL disease families that develop paraganglioma have missense VHL...

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