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Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL
von Hippel–Lindau (VHL) disease is a hereditary cancer syndrome caused by inherited mutations that inactivate the VHL tumour suppressor gene. The VHL locus encodes pVHL, whose best studied function is to bind to and down-regulate the hypoxia-inducible factor (HIF) family of oxygen-dependent transcri...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Blackwell Publishing Ltd
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3229769/ https://www.ncbi.nlm.nih.gov/pubmed/21155973 http://dx.doi.org/10.1111/j.1582-4934.2010.01238.x |
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author | Li, Mingqing Kim, William Y |
author_facet | Li, Mingqing Kim, William Y |
author_sort | Li, Mingqing |
collection | PubMed |
description | von Hippel–Lindau (VHL) disease is a hereditary cancer syndrome caused by inherited mutations that inactivate the VHL tumour suppressor gene. The VHL locus encodes pVHL, whose best studied function is to bind to and down-regulate the hypoxia-inducible factor (HIF) family of oxygen-dependent transcription factors. Early efforts have established the fundamental role of HIF in VHL-defective tumorigenesis and in particular renal cell carcinoma. However, recent findings have revealed an alternate side to the story, the HIF-independenttumour suppressor functions of pVHL. These include pVHL's ability to regulate apoptosis and senescence as well as its role in the maintenance of primary cilium and orchestrating the deposition of the extracellular matrix. To what extent these HIF-dependent and HIF-independent functions cooperate in VHL-defective tumorigenesis remains to be determined. |
format | Online Article Text |
id | pubmed-3229769 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Blackwell Publishing Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-32297692012-02-01 Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL Li, Mingqing Kim, William Y J Cell Mol Med Reviews von Hippel–Lindau (VHL) disease is a hereditary cancer syndrome caused by inherited mutations that inactivate the VHL tumour suppressor gene. The VHL locus encodes pVHL, whose best studied function is to bind to and down-regulate the hypoxia-inducible factor (HIF) family of oxygen-dependent transcription factors. Early efforts have established the fundamental role of HIF in VHL-defective tumorigenesis and in particular renal cell carcinoma. However, recent findings have revealed an alternate side to the story, the HIF-independenttumour suppressor functions of pVHL. These include pVHL's ability to regulate apoptosis and senescence as well as its role in the maintenance of primary cilium and orchestrating the deposition of the extracellular matrix. To what extent these HIF-dependent and HIF-independent functions cooperate in VHL-defective tumorigenesis remains to be determined. Blackwell Publishing Ltd 2011-02 2011-02-24 /pmc/articles/PMC3229769/ /pubmed/21155973 http://dx.doi.org/10.1111/j.1582-4934.2010.01238.x Text en © 2011 The Authors Journal of Cellular and Molecular Medicine © 2011 Foundation for Cellular and Molecular Medicine/Blackwell Publishing Ltd |
spellingShingle | Reviews Li, Mingqing Kim, William Y Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL |
title | Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL |
title_full | Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL |
title_fullStr | Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL |
title_full_unstemmed | Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL |
title_short | Two sides to every story: the HIF-dependent and HIF-independent functions of pVHL |
title_sort | two sides to every story: the hif-dependent and hif-independent functions of pvhl |
topic | Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3229769/ https://www.ncbi.nlm.nih.gov/pubmed/21155973 http://dx.doi.org/10.1111/j.1582-4934.2010.01238.x |
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