Towards a mechanistic understanding of von Hippel-Lindau syndrome in different tissues

Inactivation of the VHL tumor suppressor gene is linked to the development of several different tumor types in humans, including hemangioblastomas of the retina and central nervous system, clear cell renal carcinoma, pancreatic cysts, pheochromocytoma. Biochemical studies revealed that pVHL interacts with several different proteins serving as a multipurpose adaptor protein that engages in multiple protein-protein interactions to control diverse transcriptional-dependent and transcriptional-independent ...