Encyclopedia of Systems Biology

2013 Edition
| Editors: Werner Dubitzky, Olaf Wolkenhauer, Kwang-Hyun Cho, Hiroki Yokota


  • Bernhard Schmierer
Reference work entry
DOI: https://doi.org/10.1007/978-1-4419-9863-7_769


The tumor suppressor protein von Hippel Lindau or pVHL is part of an E3-ubiquitin ligase complex. Mutation of the protein causes von Hippel–Lindau disease, a rare genetic disorder characterized by the formation of hemangioblastomas, vasculature-derived tumors in the neurosystem.


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© Springer Science+Business Media, LLC 2013

Authors and Affiliations

  1. 1.Department of BiochemistryOxford Centre for Integrative Systems Biology (OCISB), University of OxfordOxfordUK