P40337

VHL(Von Hippel-Lindau syndrome) is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign tumors. VHL plays a crucial role as a tumor suppressor, and dysfunction is linked to von Hippel-Lindau syndrome, which predisposes individuals to several tumor types, including renal cell carcinoma, hemangioblastomas, and pheochromocytomas. VHL protein is primarily located in the cytoplasm and nucleus, where VHL interacts with the Elongin (SIII) complex, a heterotrimeric protein complex essential for regulating gene transcription. By binding to the Elongin B and C subunits, VHL protein inhibits the transcriptional activity of this complex, thereby playing a vital role in cellular processes such as oxygen sensing and the regulation of hypoxia-inducible factors. Different isoforms of VHL protein, resulting from alternatively spliced transcript variants, highlight the complexity of regulation and function, with variations in molecular weight observed across species.