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vhl
VHL
VHL tumor suppressor targets HIF-1α for proteasomal degradation under normoxia; VHL loss → constitutive HIF activation → VEGF, PDGF, and EPO. Biallelic VHL inactivation causes clear cell RCC (~75%) and von Hippel-Lindau syndrome (hemangioblastomas, pheochromocytoma).
Entry Metadata
| Field | Value |
|---|---|
| ID | vhl |
| Name | VHL |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 03-molecular |
Cross-Atlas Connections
Sources
- Kaelin WG Jr. The von Hippel-Lindau tumour suppressor protein: O2 sensing and cancer. Nat Rev Cancer. 2008;8(11):865-873. · PubMed 18923434
- Choueiri TK, Bauer TM, Papadopoulos KP, et al. Inhibition of hypoxia-inducible factor-2alpha in renal cell carcinoma with belzutifan. N Engl J Med. 2021;385(22):2059-2071. · PubMed 34818478