Article
The Endothelial Landscape and Its Role in Von Hippel-Lindau Disease.
Cells - 4 Sept 2021
de Rojas-P Isabel, Albiñana Virginia, Taranets Lyudmyla, Recio-Poveda Lucía, Cuesta Angel M, Popov Nikita, Kronenberger Thales, Botella Luisa M
Abstract excerpt
Von Hippel-Lindau disease (VHL) is a rare hereditary disease characterized by the predisposal to develop different types of highly vascularized tumors. VHL patients carry a VHL mutation that causes partial lack of functional VHL protein (pVHL) in all cells, and a total lack thereof in cells harboring a second hit mutation. Absence of pVHL generates a prolonged state of pseudo-hypoxia in the cell due to...
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