What is VHL in HIF?
Under conditions of normal oxygen tension, the HIF-alpha subunit is hydroxylated by specific prolyl-hydroxylases and targeted for rapid proteasomal degradation by the von Hippel-Lindau (VHL) tumor suppressor, which is the substrate recognition component of an E3-ubiquitin ligase.
Is VHL a tumor suppressor?
The VHL protein likely plays a role in other cellular functions, including the regulation of other genes and control of cell division. Based on this function, the VHL protein is classified as a tumor suppressor, which means it prevents cells from growing and dividing too rapidly or in an uncontrolled way.
What does VHL stand for?
Von Hippel-Lindau syndrome (VHL) is a hereditary condition associated with tumors arising in multiple organs. VHL-related tumors include hemangioblastomas, which are blood vessel tumors of the brain, spinal cord, and retina.
Is VHL a E3 ubiquitin ligase?
Von Hippel-Lindau (VHL) is an E3 ubiquitin ligase that targets proteins, including HIF-1α, for proteasomal degradation. VHL and HIF regulate the balance between glycolysis and oxidative phosphorylation, which is critical in highly dynamic T cells.
Where is VHL located?
The VHL gene is a tumor suppressor gene located on chromosome 3, which usually controls cell growth and cell death.
How is VHL regulated?
VHL proteostasis is regulated by multiple mechanisms including folding, chaperone binding, complex formation and phosphorylation.
Is VHL curable?
Von Hippel-Lindau disease has no cure. The goal of treatment is to find and remove tumors as early as possible, before they affect your health. Your provider may recommend surgery to remove tumors whenever possible. Some tumors may respond to radiation therapy.
Who discovered Von Hippel-Lindau?
Arvid Lindau, MD, PhD, consolidated the disparate array of benign and malignant visceral and nervous system lesions into the neoplastic syndrome known as von Hippel-Lindau (VHL) disease.
What is the VHL hockey?
The VHL (the “Vysshaya Khokkeinaya Liga” or “Higher Hockey League”) is the second tier of Russian men’s professional hockey, formerly the second division of the Soviet and Russian championship system back when promotion and relegation took place.
Do I have VHL?
The only way to confirm VHL is by genetic testing to see if you have the gene mutation. If anyone in your family has Von Hippel-Lindau disease, ask your healthcare provider if genetic testing is right for you. Healthcare providers often diagnose VHL after someone has symptoms related to a VHL tumor.
What is VHL protac?
The proteolysis targeting chimera (PROTAC) technology has recently emerged as a powerful approach for inducing protein degradation. A potent and orally active VHL-recruiting PROTAC was developed, displaying robust HMGCR degradation and potent hypolipidemic activity in diet-induced mice.
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