نتایج جستجو برای: vhl

تعداد نتایج: 1947  

2016
Victoria Schunemann Kristin Huntoon Russell R. Lonser

von Hippel-Lindau disease (VHL) is a familial neoplasia syndrome associated with multisystem tumor development. Depending on tumor type and location, current treatments for VHL-associated tumors can include a combination of chemotherapy, radiation therapy, and/or surgery. Central nervous system (CNS) manifestations of VHL include craniospinal hemangioblastomas and endolymphatic sac tumors (ELST...

2014
Boban Stanojevic Vladimir Saenko Lidija Todorovic Nina Petrovic Dragan Nikolic Vladan Zivaljevic Ivan Paunovic Masahiro Nakashima Shunichi Yamashita Radan Dzodic

Alterations of the von Hippel-Lindau (VHL) tumor suppressor gene can cause different hereditary tumors associated with VHL syndrome, but the potential role of the VHL gene in papillary thyroid carcinoma (PTC) has not been characterized. This study set out to investigate the relationship of VHL expression level with clinicopathological features of PTC in an ethnically and geographically homogeno...

2018
Susan E. Scanlon Denise C. Hegan Parker L. Sulkowski Peter M. Glazer

The von Hippel-Lindau (VHL) tumor suppressor gene is inactivated in the vast majority of human clear cell renal carcinomas. The pathogenesis of VHL loss is currently best understood to occur through stabilization of the hypoxia-inducible factors, activation of hypoxia-induced signaling pathways, and transcriptional reprogramming towards a pro-angiogenic and pro-growth state. However, hypoxia al...

Journal: :Molecular and cellular biology 2007
Andrew J Evans Ryan C Russell Olga Roche T Nadine Burry Jason E Fish Vinca W K Chow William Y Kim Arthy Saravanan Mindy A Maynard Michelle L Gervais Roxana I Sufan Andrew M Roberts Leigh A Wilson Mark Betten Cindy Vandewalle Geert Berx Philip A Marsden Meredith S Irwin Bin T Teh Michael A S Jewett Michael Ohh

The product of the von Hippel-Lindau gene (VHL) acts as the substrate-recognition component of an E3 ubiquitin ligase complex that ubiquitylates the catalytic alpha subunit of hypoxia-inducible factor (HIF) for oxygen-dependent destruction. Although emerging evidence supports the notion that deregulated accumulation of HIF upon the loss of VHL is crucial for the development of clear-cell renal ...

Journal: :American journal of physiology. Renal physiology 2004
Midori Yamakawa Louis X Liu Adam J Belanger Taro Date Takayuki Kuriyama Mark A Goldberg Seng H Cheng Richard J Gregory Canwen Jiang

The hereditary von Hippel-Lindau (VHL) syndrome predisposes sufferers to highly vascularized tumors such as renal clear cell carcinoma (RCC) and central nervous system hemangioblastoma. In RCC4 and RCC786-0 VHL- cells with VHL mutations, the protein of hypoxia-inducible factor-1alpha (HIF-1alpha) is constitutively stabilized and the mRNA levels of HIF target genes, including vascular endothelia...

Journal: :Canadian journal of ophthalmology. Journal canadien d'ophtalmologie 2005
Craig Moskowitz Chaim Edelstein Mila Oh Miguel N Burnier

von Hippel-Lindau (VHL) disease is caused by a mutation in the VHL gene, resulting in the functional disorder in VHL-encoded protein (pVHL). Recent advances in experimental and clinical studies on VHL gene/protein and VHL disease have provided novel concepts in molecular pathology and clinical managements. pVHL plays a critical role in the regulation of hypoxia inducible factor (HIF)-dependent ...

Journal: :Diabetes 2009
Sapna Puri David A. Cano Matthias Hebrok

OBJECTIVE The Vhlh gene codes for the von Hippel-Lindau protein (VHL), a tumor suppressor that is a key player in the cellular response to oxygen sensing. In humans, a germline mutation in the VHL gene leads to the von Hippel-Lindau disease, a familial syndrome characterized by benign and malignant tumors of the kidney, central nervous system, and pancreas. RESEARCH DESIGN AND METHODS We use ...

2011
Federico Formenti Philip A. Beer Quentin P. P. Croft Keith L. Dorrington Daniel P. Gale Terence R. J. Lappin Guy S. Lucas Eamonn R. Maher Patrick H. Maxwell Mary F. McMullin David F. O'Connor Melanie J. Percy Christopher W. Pugh Peter J. Ratcliffe Thomas G. Smith Nick P. Talbot Peter A. Robbins

The hypoxia-inducible factors (HIFs; isoforms HIF-1α, HIF-2α, HIF-3α) mediate many responses to hypoxia. Their regulation is principally by oxygen-dependent degradation, which is initiated by hydroxylation of specific proline residues followed by binding of von Hippel-Lindau (VHL) protein. Chuvash polycythemia is a disorder with elevated HIF. It arises through germline homozygosity for hypomorp...

2002
Hidetoshi Murata Nobuyoshi Tajima Yoji Nagashima Masahiro Yao Masaya Baba Masayuki Goto Susumu Kawamoto Isao Yamamoto Kenji Okuda Hiroshi Kanno

Von Hippel-Lindau (VHL) tumor suppressor protein is expressed in neurons of the central nervous system and plays an important role during the neuronal differentiation of central nervous system progenitor cells. To elucidate the neuronal differentiating potential of VHL protein in neuroblastoma cells, we overexpressed or inhibited VHL protein in human neuroblastoma cells (SY-SH5Y), and examined ...

2017
Bum Jun Kim Jung Han Kim Hyeong Su Kim Dae Young Zang

The von Hippel-Lindau (VHL) gene is often inactivated in sporadic renal cell carcinoma (RCC) by mutation or promoter hypermethylation. The prognostic or predictive value of VHL gene alteration is not well established. We conducted this meta-analysis to evaluate the association between the VHL alteration and clinical outcomes in patients with RCC. We searched PUBMED, MEDLINE and EMBASE for artic...

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