作者: Michael Ohh , William G Kaelin , William Kaelin
DOI: 10.1016/S1357-4310(99)01481-1
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摘要: von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome caused by germline mutations of the VHL tumour suppressor gene. The gene product, pVHL, forms multiprotein complexes that contain elongin B, C and Cul-2, negatively regulates hypoxia-inducible mRNAs. pVHL suspected to play role in ubiquitination given similarity Cul-2 with Skp1 Cdc53, respectively. can also interact fibronectin required for assembly matrix. Finally, at least indirectly, plays ability cells exit cell cycle. Thus, protein angiogenesis, extracellular matrix formation