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TranscriptomicsRNA expression data from glomeruli lacking von Hippel-Lindau protein in podocytes
We and others have previously shown that glomerular endothelial cells and podocytes express hypoxia-inducible transcription factors (HIFs). HIFs bind to hypoxia response elements in target genes, such as vascular endothelial growth factor, which is continually produced by podocytes throughout life....
ORGANISM(S): Mus musculus 
2010-08-03 | E-GEOD-20235 | biostudies-arrayexpress
GenomicsVon Hippel-Lindau syndrome multi-region exome sequencing project from two patients undertaken at Cancer Research UK's London Research Institute
Multi-region whole exome sequencing of tumours from patients with Von Hippel-Lindau syndrome reveals convergence of mutayional events upon the PI3L-AKT-mTOR signalling pathway.
EGAS00001000907 | EGA
TranscriptomicsGenetic Analysis of Pathways Regulated by the von Hippel-Lindau Tumor Suppressor in Caenorhabditis elegans
The von Hippel-Lindau (VHL) tumor suppressor functions as a ubiquitin ligase that mediates proteolytic inactivation of hydroxylated a subunits of hypoxia-inducible factor (HIF). Although studies of VHL defective renal carcinoma cells suggest the existence of other VHL tumor suppressor pathways, dysr...
ORGANISM(S): Caenorhabditis elegans 
2005-07-20 | E-GEOD-2963 | biostudies-arrayexpress
TranscriptomicsIGR_VHL_GE
VHL is a tumor suppressor gene involved in the oxygen-sensing pathway whose germline mutations predispose to distinct phenotypes. Heterozygous mutations predispose to von Hippel-Lindau disease characterized by the development of multiple tumors (including hemangioblastomas, renal cell carcinomas and...
ORGANISM(S): Homo sapiens 
2013-09-03 | E-MTAB-1269 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of liver from mice with constitutively active Hypoxia Inducible Factor (HIF-1a and HIF-2a) or inactivated von Hippel-Lindau tumor suppressor gene product (pVHL)
The effects of constitutively active Hypoxia Inducible Factor (HIF) and inactivated von Hippel-Lindau tumor suppressor gene product (pVHL) were examined in a mouse model. Conditionally expressed, constitutively active HIF-1a and HIF-2a were compared with inactivated pVHL.
ORGANISM(S): Mus musculus 
2006-09-21 | E-MEXP-856 | biostudies-arrayexpress
TranscriptomicsPleiotropic Effects of the Trichloroethylene-Associated P81S VHL Mutation on Metabolism, Apoptosis and ATM-Mediated DNA Damage Response
Gene expression data from VHL teratomas comparing genes differentially expressed based on apoptotic response to tumor microenvironment. Abstract BACKGROUND: The risk relevance of the P81S von Hippel-Lindau (VHL) gene hotspot mutation identified in clear cell renal cell carcinoma from individuals ex...
ORGANISM(S): Mus musculus 
2013-09-01 | E-GEOD-37464 | biostudies-arrayexpress
TranscriptomicsPatterns of gene expression and copy-number alterations in VHL disease-associated and sporadic ccRCC
Recent insights into the role of the VHL tumor suppressor gene in hereditary and sporadic clear cell carcinoma of the kidney (ccRCC) have led to new treatments for patients with metastatic ccRCC, although virtually all patients eventually succumb to the disease. We performed an integrated, genome-wi...
ORGANISM(S): Homo sapiens 
2010-04-10 | E-GEOD-14994 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of human VHL treated A498 renal cancer cells
A498 cells, established from a clear cell renal cell cancer of a 52 years old male patient, were obtained from ATCC and maintained in DMEM supplemented with 10% fetal calf serum (FCS). This cell line does not express functional VHL. A498 subclones stably transfected with HA-pVHL30 or control plasmid...
ORGANISM(S): Homo sapiens 
2003-09-18 | E-MEXP-13 | biostudies-arrayexpress
ProteomicsOxygen sensitive regulation of TFAM linking the von Hippel-Lindau cancer syndrome to impaired mitochondrial biogenesis
The major consumers of oxygen in the cell are mitochondria. Here we identified an oxygen-sensitive regulation of TFAM, a key activator of mitochondrial transcription and replication. TFAM is hydroxylated by EglN3 on proline 53/56 and subsequently bound by the tumor suppressor von Hippel Lindau (pVHL...
ORGANISM(S): Homo sapiens (Human) 
2022-05-25 | PXD022734 | Pride
Lc-ms/ms Tmt Mitocondria Hippel-lindau cancer syndrome Oxygen regulation 
TranscriptomicsArray CGH analysis in primary human renal tumors, categorized by VHL genotype and HIF-alpha expression
Human renal cell carcinomas (RCC) have differential expression of HIF-1alpha and HIF-2alpha, depending on VHL genotype and other events. Here, we have divided a series of RCC samples based on HIF-alpha expression, in order to examine levels of genomic DNA aberration. Keywords: Patient Sample Study ...
ORGANISM(S): Homo sapiens 
2008-12-27 | E-GEOD-13282 | biostudies-arrayexpress
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