The DOI matches its Crossref record; 9 of the 9 references it cites were found in a registry or an index.
Formation of Primary Cilia in the Renal Epithelium Is Regulated by the von Hippel-Lindau Tumor Suppressor Protein
Abstract
Growing evidence points to defects in the primary cilium as a critical mechanism underlying renal cyst development. Inactivation of the VHL gene is responsible for the autosomal dominant condition von Hippel-Lindau (VHL) disease and is implicated in most sporadic clear cell renal carcinomas. Manifestations of VHL disease include cysts in several organs, particularly in the kidney. Here it is shown that VHL inactivation is associated with abrogation of the primary cilium in renal cysts of patients with VHL disease and in VHL-defective cell lines. Complementation of VHL-defective clear cell renal carcinoma cell lines with wild-type VHL restored primary cilia. Moreover, it is shown that the effects of VHL on the primary cilium are mediated substantially via hypoxia-inducible factor. The effect of VHL status on the primary cilium provides a potential mechanism for renal cyst development in VHL disease and may help in the understanding of how VHL acts as a tumor suppressor.
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Cites
Targeting HIF-1 for cancer therapy
Semenza, G. L. (2003) · Nature Reviews Cancer · doi:10.1038/nrc1187 · Cited by 6,717
The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysis
Maxwell, P. H., Wiesener, M. S., Chang, G.-W., Clifford, S. C., Vaux, E. C., Cockman, M. E., Wykoff, C. C., Pugh, C. W., Maher, E. R., & Ratcliffe, P. J. (1999) · Nature · doi:10.1038/20459 · Cited by 5,180
Self-citationPolycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells
Nauli, S. M., Alenghat, F. J., Luo, Y., Williams, E., Vassilev, P., Li, X., Elia, A. E. H., Lu, W., Brown, E. M., Quinn, S. J., et al. (2003) · Nature Genetics · doi:10.1038/ng1076 · Cited by 2,057
Pazour, G. J., Dickert, B. L., Vucica, Y., Seeley, E. S., Rosenbaum, J. L., Witman, G. B., & Cole, D. G. (2000) · The Journal of Cell Biology · doi:10.1083/jcb.151.3.709 · Cited by 1,147
Raval, R. R., Lau, K. W., Tran, M. G. B., Sowter, H. M., Mandriota, S. J., Li, J.-L., Pugh, C. W., Maxwell, P. H., Harris, A. L., & Ratcliffe, P. J. (2005) · Molecular and Cellular Biology · doi:10.1128/mcb.25.13.5675-5686.2005 · Cited by 940
Self-citationMolecular basis of the VHL hereditary cancer syndrome
Kaelin, W. G. (2002) · Nature Reviews Cancer · doi:10.1038/nrc885 · Cited by 865
Inhibition of HIF is necessary for tumor suppression by the von Hippel-Lindau protein
Kondo, K., Klco, J., Nakamura, E., Lechpammer, M., & Kaelin, W. G. (2002) · Cancer Cell · doi:10.1016/s1535-6108(02)00043-0 · Cited by 761
Clifford, S. C. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.10.1029 · Cited by 373
Regulation of E-cadherin Expression by VHL and Hypoxia-Inducible Factor
Esteban, M. A., Tran, M. G. B., Harten, S. K., Hill, P., Castellanos, M. C., Chandra, A., Raval, R., O'Brien, T. S., & Maxwell, P. H. (2006) · Cancer Research · doi:10.1158/0008-5472.can-05-2670 · Cited by 262
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Cited by
When cilia go bad: cilia defects and ciliopathies
Fliegauf, M., Benzing, T., & Omran, H. (2007) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm2278 · Cited by 1,257
VHL and HIF signalling in renal cell carcinogenesis
Baldewijns, M. M., van Vlodrop, I. J., Vermeulen, P. B., Soetekouw, P. M., van Engeland, M., & de Bruïne, A. P. (2010) · The Journal of Pathology · doi:10.1002/path.2689 · Cited by 316
The VHL tumor suppressor: master regulator of HIF
Haase, V. (2009) · Current Pharmaceutical Design · doi:10.2174/138161209789649394 · Cited by 175
The VHL tumor suppressor and HIF: insights from genetic studies in mice
Kapitsinou, P. P., & Haase, V. H. (2008) · Cell Death & Differentiation · doi:10.1038/sj.cdd.4402313 · Cited by 151
Borgal, L., Habbig, S., Hatzold, J., Liebau, M. C., Dafinger, C., Sacarea, I., Hammerschmidt, M., Benzing, T., & Schermer, B. (2012) · Journal of Biological Chemistry · doi:10.1074/jbc.m112.385658 · Cited by 56
Metadata
- Authors
- Esteban, M. A., Harten, S. K., Tran, M. G., & Maxwell, P. H.
- Venue
- Journal of the American Society of Nephrology
- Published
- 2006-07
- Volume
- 17
- Issue
- 7
- Pages
- 1801-1806
- ISSN
- 1046-6673 (ISSN-L)
- OpenAlex
- W2165295267
- Cited by
- 161
- References
- 9
- FWCI
- 8.66
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Formation of Primary Cilia in the Renal Epithelium Is Regulated by the von Hippel-Lindau Tumor Suppressor Protein. https://app.cochat.ai/papers/10.1681/asn.2006020181
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- Esteban, M. A., Harten, S. K., Tran, M. G., & Maxwell, P. H. (2006). Formation of Primary Cilia in the Renal Epithelium Is Regulated by the von Hippel-Lindau Tumor Suppressor Protein. Journal of the American Society of Nephrology. https://doi.org/10.1681/asn.2006020181
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