The VHL gene provides instructions for making a [[protein]] that functions as part of a complex (a group of proteins that work together) called the VCB-CUL2 complex. This complex targets other proteins to be broken down (degraded) by the cell when they are no longer needed.

[[Hemangioblastoma]]s may arise sporadically in a solitary form or associated with [[Von Hippel-Lindau disease]] with multiple tumors. 
[[VHL protein]] is defective in both forms of [[hemangioblastoma]], leading to the accumulation of hypoxia-inducible factor, stimulating [[angiogenesis]] via [[VEGF]] and [[PDGF]] mainly
((Migliorini D, Haller S, Merkler D, Pugliesi-Rinaldi A, Koka A, Schaller K, Leemann B, Dietrich PY. Recurrent multiple CNS hemangioblastomas with VHL disease treated with pazopanib: a case report and literature review. CNS Oncol. 2015;4(6):387-92. doi: 10.2217/cns.15.22. Epub 2015 Oct 26. PMID: 26497655; PMCID: PMC6083944.)).