Models of Von Hippel-Lindau tumor suppressor disease specific activity.


9553 Background: The Von Hippel-Lindau (VHL) syndrome is an autosomal dominant disorder characterized by mutations in a classical tumor suppressor gene (VHL). Clear cell renal cell carcinoma and hemangioblastoma without pheochromocytoma characterize VHL type 1 disease. VHL type 2 disease is characterized by the presence of pheochromocytoma and is further… (More)