VHL and von Hippel-Lindau disease: However, mutation of pVHL severely destabilizes the binding of pVHL to elonginB/C, leading to ubiquitination and degradation of pVHL; when pVHL is degraded or absent, HIF-α accumulates in the nucleus and subsequently promotes various tumors by activating the transcription of various oncogenic molecules, which is manifested as the VHL syndrome [92–94].