@article{c7c1b10b29544bdd87dfad67a897895b,
title = "The von hippel-lindau tumor suppressor gene: Implications and therapeutic opportunities",
abstract = "The discovery of the von Hippel-Lindau (VHL) gene marked a milestone in our understanding of clear cell renal cell carcinoma (ccRCC) pathogenesis. VHL inactivation is not only a defining feature of ccRCC, but also the initiating event. Herein, we discuss canonical and noncanonical pVHL functions, as well as breakthroughs shaping our understanding of ccRCC evolution and evolutionary subtypes. We conclude by presenting evolving strategies to therapeutically exploit effector mechanisms downstream of pVHL.",
keywords = "AROHIF2, MK-6482, PT2385, PT2977, hypoxia-inducible factor, siRNA, synthetic lethality, tumor evolution",
author = "Roy Elias and Qing Zhang and James Brugarolas",
note = "Funding Information: Conflicts of Interest and Source of Funding: J.B. reports personal fees from Arrowhead Pharmaceuticals, personal fees from Exilixis Pharmaceuticals, outside the submitted work. In addition, J.B. has US Patent No. 15/761,534 issued, and two provisional patent applications. J.B. receives support from NIH SPORE grant P50CA196516 and Cancer Prevention & Research Institute of Texas (CPRIT) grant RP130603. Q.Z. is supported by DoD Kidney Cancer Research Program idea development award (W81XWH1910813), the kidney cancer research alliance, CPRIT grant RR190058, American Cancer Society grant RSG-18-059-01-TBE, and NCI grant R01CA211732. R.E. receives support from an institutional award provided through the Burroughs Wellcome Fund. Publisher Copyright: {\textcopyright} Wolters Kluwer Health, Inc. All rights reserved.",
year = "2020",
month = sep,
day = "1",
doi = "10.1097/PPO.0000000000000480",
language = "English (US)",
volume = "26",
pages = "390--398",
journal = "Cancer Journal",
issn = "1528-9117",
number = "5",
}