The von Hippel–Lindau tumour suppressor protein: new perspectives

作者: Michael Ohh , William G Kaelin , William Kaelin

DOI: 10.1016/S1357-4310(99)01481-1

关键词:

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

参考文章(50)
T. Aso, D. Haque, R. J. Barstead, R. C. Conaway, J. W. Conaway, The inducible elongin A elongation activation domain: structure, function and interaction with the elongin BC complex. The EMBO Journal. ,vol. 15, pp. 5557- 5566 ,(1996) , 10.1002/J.1460-2075.1996.TB00940.X
I A Lubensky, J R Gnarra, W M Linehan, Z Zhuang, M M Walther, P Bertheau, Allelic deletions of the VHL gene detected in multiple microscopic clear cell renal lesions in von Hippel-Lindau disease patients American Journal of Pathology. ,vol. 149, pp. 2089- 2094 ,(1996)
Othon Iliopoulos, Janusz Rak, Serge Jothy, William Kaelin, Blandine Lieubeau-Teillet, Robert S. Kerbel, von Hippel-Lindau Gene-mediated Growth Suppression and Induction of Differentiation in Renal Cell Carcinoma Cells Grown as Multicellular Tumor Spheroids Cancer Research. ,vol. 58, pp. 4957- 4962 ,(1998)
Kim M. Lonergan, Othon Iliopoulos, Michael Ohh, Takumi Kamura, Ronald C. Conaway, Joan Weliky Conaway, William G. Kaelin, Regulation of Hypoxia-Inducible mRNAs by the von Hippel-Lindau Tumor Suppressor Protein Requires Binding to Complexes Containing Elongins B/C and Cul2 Molecular and Cellular Biology. ,vol. 18, pp. 732- 741 ,(1998) , 10.1128/MCB.18.2.732
Patricia M. Kessler, Sandip P. Vasavada, Raymond R. Rackley, Thomas Stackhouse, Fuh-Mei Duh, Farida Latif, Michael I. Lerman, Berton Zbar, Bryan R. G. Williams, Expression of the Von Hippel-Lindau tumor suppressor gene, VHL, in human fetal kidney and during mouse embryogenesis. Molecular Medicine. ,vol. 1, pp. 457- 466 ,(1995) , 10.1007/BF03401583
Ron R Kopito, ER quality control: the cytoplasmic connection. Cell. ,vol. 88, pp. 427- 430 ,(1997) , 10.1016/S0092-8674(00)81881-4
O. Iliopoulos, M. Ohh, W. G. Kaelin, pVHL19 is a biologically active product of the von Hippel–Lindau gene arising from internal translation initiation Proceedings of the National Academy of Sciences of the United States of America. ,vol. 95, pp. 11661- 11666 ,(1998) , 10.1073/PNAS.95.20.11661
O. Iliopoulos, A. P. Levy, C. Jiang, W. G. Kaelin, M. A. Goldberg, Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau protein Proceedings of the National Academy of Sciences of the United States of America. ,vol. 93, pp. 10595- 10599 ,(1996) , 10.1073/PNAS.93.20.10595
A. Pause, S. Lee, R. A. Worrell, D. Y. T. Chen, W. H. Burgess, W. M. Linehan, R. D. Klausner, The von Hippel-Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteins Proceedings of the National Academy of Sciences of the United States of America. ,vol. 94, pp. 2156- 2161 ,(1997) , 10.1073/PNAS.94.6.2156
T. Kamura, S. Sato, D. Haque, L. Liu, W. G. Jr. Kaelin, R. C. Conaway, J. W. Conaway, The Elongin BC complex interacts with the conserved SOCS-box motif present in members of the SOCS, ras, WD-40 repeat, and ankyrin repeat families Genes & Development. ,vol. 12, pp. 3872- 3881 ,(1998) , 10.1101/GAD.12.24.3872