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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. The Von Hippel–Lindau tumor suppressor also known as pVHL is a protein that, in humans, is encoded by the VHL gene. The VHL protein functions as a subunit of a multiprotein ubiquitin ligase that negatively regulates expression of a large collection of hypoxia-inducible genes. Von Hippel-Lindau syndrome (VHL) is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign tumors. This protein is involved in the pathway protein ubiquitination, which is part of Protein modification. von Hippel-Lindau disease tumor suppressor.