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https://www.readbyqxmd.com/read/28212363/vegf-inhibitors-in-renal-cell-carcinoma
#1
Pankit Vachhani, Saby George
The arrival of targeted therapies-vascular endothelial growth factor (VEGF) pathway inhibitors and mammalian target of rapamycin (mTOR) inhibitors-and programmed death 1 (PD-1) inhibitors has transformed the management of renal cell carcinoma (RCC). Once considered fatal, with a median survival of approximately 1 year, these agents have nearly tripled overall survival and have raised hopes of a possible cure for advanced RCC. This review begins with a brief discussion of the seminal von Hippel-Lindau/hypoxia-inducible factor axis in RCC...
December 2016: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/28196363/a-paratesticular-multicystic-tumor-of-the-tunica-vaginalis-testis-as-rare-paratesticular-cystadenoma
#2
Desiree Louise Draeger, Stine-Kathrein Kraeft, Chris Protzel, Oliver W Hakenberg
The cystadenoma of the testis and paratestis arising from an unequivocal oviduct-like structure, which is morphologically almost identical with those of the ovarian surface epithelium. These are very rare benign tumors of young adults. They present as asymptomatic cystic lesions. Bilateral paratesticular cystadenomas are strongly associated with von Hippel-Lindau syndrome and correlate with infertility. It is a neoplasm with low malignant potential. Most cystadenomas are benign but a few cases of malignant transformation of embryonic remnants have been reported in the appendix testis, including cases of adenocarcinoma, cystadenocarcinoma, and a low malignant müllerian-type epithelial tumor...
February 15, 2017: Urologia Internationalis
https://www.readbyqxmd.com/read/28179177/advantage-of-high-b-value-diffusion-weighted-imaging-for-differentiation-of-hemangioblastoma-from-brain-metastases-in-posterior-fossa
#3
Shumpei Onishi, Takanori Hirose, Takeshi Takayasu, Ryo Nosaka, Manish Kolakshyapati, Taiichi Saito, Yuji Akiyama, Kazuhiko Sugiyama, Kaoru Kurisu, Fumiyuki Yamasaki
OBJECTIVE: It is sometimes difficult to make differential diagnosis between brain metastases and hemangioblastoma in posterior fossa. We assessed whether high b-value diffusion weighted image (DWI) at b-4000 could differentiate these tumors. METHODS: We acquired DWI at 3T magnetic resonance imaging (MRI) with b = 1000 and b = 4000 s/mm(2) in histologically confirmed 12 patients of hemangioblastoma without von-Hippel Lindau disease and 16 patients of brain metastases originating at posterior fossa...
February 4, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28175584/367%C3%A2-autocrine-paracrine-erythropoietin-signaling-associated-with-symptomatic-von-hippel-lindau-hemangioblastomas
#4
Saman Sizdahkhani, Xiang Wang, Nancy A Edwards, Zhengping Zhuang, Russell R Lonser, Edward H Oldfield, Prashant Chittiboina
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
https://www.readbyqxmd.com/read/28169069/difficulties-in-the-management-of-retinal-capillary-haemangiomas-associated-with-von-hippel-lindau-disease
#5
M Pastor-Montoro, N Hurtado-Montalbán, J A Martínez-Morales, M P Villegas-Pérez
CLINICAL CASE: A 29-year-old female with bilateral retinal capillary haemangiomas (RCH). A genetic analysis was carried out due to the suspicion of von Hippel-Lindau (VHL) disease, with negative results on 2occasions. There was progression of the RCH in the left eye, leading to a macular epiretinal membrane. The patient was treated with laser, intravitreal ranibizumab, and vitrectomy. Finally, a third genetic test detected a de novo mutation in the VHL gene, and led to the genetic diagnosis...
February 3, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28149884/p53-mutations-and-cancer-a-tight-linkage
#6
COMMENT
Francesco Perri, Salvatore Pisconti, Giuseppina Della Vittoria Scarpati
P53 is often mutated in solid tumors, in fact, somatic changes involving the gene encoding for p53 (TP53) have been discovered in more than 50% of human malignancies and several data confirmed that p53 mutations represent an early event in cancerogenesis. Main p53 functions consist in cell cycle arrest, DNA repair, senescence and apoptosis induction in response to mutagenic stimuli, and, to exert those functions, p53 acts as transcriptional factor. Recent data have highlighted another very important role of p53, consisting in regulate cell metabolism and cell response to oxidative stress...
December 2016: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28129856/zebrafish-as-a-model-for-von-hippel-lindau-and-hypoxia-inducible-factor-signaling
#7
H R Kim, D Greenald, A Vettori, E Markham, K Santhakumar, F Argenton, F van Eeden
Oxygen is a central molecule in the development of multicellular life, allowing efficient energy generation. Inadequate oxygen supply requires rapid adaptations to prevent cellular damage and the hypoxia-inducible factor (HIF) pathway plays a central role in this adaptation. Numerous diseases and disease processes are influenced by hypoxia and the HIF pathway. One component, von Hippel Lindau (VHL), is a well-known tumor suppressor, which acts at least in part via regulating HIF signaling. The zebrafish has become a central vertebrate model organism in which developmental and disease processes can be studied...
2017: Methods in Cell Biology
https://www.readbyqxmd.com/read/28129633/stereotactic-radiosurgery-versus-surgical-resection-for-spinal-hemangioblastoma-a-systematic-review
#8
REVIEW
Kelly J Bridges, Jerry J Jaboin, Charlotte D Kubicky, Khoi D Than
Spinal cord hemangioblastomas are benign vascular tumors arising sporadically in approximately 70-80% of cases. They can also be manifestations of von Hippel-Lindau (VHL) disease, as these patients will often have multiple spinal hemangioblastomas. Historically, surgical management of symptomatic intramedullary hemangioblastomas has been considered the treatment of choice. However, recently, stereotactic radiosurgery has been utilized as an adjuvant therapeutic modality, and some have suggested it may have utility as the primary treatment option for these tumors...
January 18, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28118605/boosting-the-hypoxic-response-in-myeloid-cells-accelerates-resolution-of-fibrosis-and-regeneration-of-the-liver-in-mice
#9
Chahrazade Kantari-Mimoun, Ewelina Krzywinska, Magali Castells, Corinne Milien, Ralph Klose, Anna-Katharina Meinecke, Ursula Lemberger, Thomas Mathivet, Milos Gojkovic, Katrin Schrödter, Christoph Österreicher, Joachim Fandrey, Helene Rundqvist, Christian Stockmann
We have recently shown that targeting Vascular Endothelial Growth Factor (VEGF) specifically in scar-infiltrating myeloid cells prevented remodeling of the sinusoidal vasculature and abrogated the resolution of murine liver fibrosis, thereby unmasking an unanticipated link between angiogenesis and resolution of fibrosis. In a gain of function approach, we wanted to test the impact of VEGF overexpression in myeloid cells on fibrolysis. We observe that genetic inactivation of the von Hippel Lindau protein (VHL), a negative regulator of Hypoxia-inducible factors (HIF) in myeloid cells, leads to increased VEGF expression and most importantly, accelerated matrix degradation and reduced myofibroblast numbers after CCl4 challenge...
January 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/28114281/ubiquitination-and-regulation-of-aurka-identifies-a-hypoxia-independent-e3-ligase-activity-of-vhl
#10
E Hasanov, G Chen, P Chowdhury, J Weldon, Z Ding, E Jonasch, S Sen, C L Walker, R Dere
The hypoxia-regulated tumor-suppressor von Hippel-Lindau (VHL) is an E3 ligase that recognizes its substrates as part of an oxygen-dependent prolyl hydroxylase (PHD) reaction, with hypoxia-inducible factor α (HIFα) being its most notable substrate. Here we report that VHL has an equally important function distinct from its hypoxia-regulated activity. We find that Aurora kinase A (AURKA) is a novel, hypoxia-independent target for VHL ubiquitination. In contrast to its hypoxia-regulated activity, VHL mono-, rather than poly-ubiquitinates AURKA, in a PHD-independent reaction targeting AURKA for degradation in quiescent cells, where degradation of AURKA is required to maintain the primary cilium...
January 23, 2017: Oncogene
https://www.readbyqxmd.com/read/28112200/reduction-of-hepatic-fibrosis-by-overexpression-of-von-hippel-lindau-protein-in-experimental-models-of-chronic-liver-disease
#11
Jizhou Wang, Zhaoyang Lu, Zhilin Xu, Pei Tian, Hui Miao, Shangha Pan, Ruipeng Song, Xueying Sun, Baolei Zhao, Dawei Wang, Yong Ma, Xuan Song, Shugeng Zhang, Lianxin Liu, Hongchi Jiang
Hypoxia-inducible factor (HIF)-1α and HIF-2α play an important role in liver fibrosis. von Hippel-Lindau protein (VHL), a key mediator of HIF-α, regulates fibrosis in an organ- and cell-specific way. In this study, human liver samples were collected from hepatitis C-, alcoholic-, and cholestatic-associated fibrotic and healthy individuals. Two mouse models of liver fibrosis were established: bile duct ligation and carbon tetrachloride injection. We constructed adenovirus vectors to overexpress VHL, normoxia-active HIF-α, and lentiviral vectors to silence HIF-α...
January 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28110220/hypoxia-inducing-factor-hif-1%C3%AE-derived-peptide-capable-of-inducing-cancer-reactive-cytotoxic-t-lymphocytes-from-hla-a24-patients-with-renal-cell-carcinoma
#12
Takafumi Minami, Naoki Matsumura, Koichi Sugimoto, Nobutaka Shimizu, Marco De Velasco, Masahiro Nozawa, Kazuhiro Yoshimura, Nanae Harashima, Mamoru Harada, Hirotsugu Uemura
Hypoxic tumor microenvironment makes cancer cells to be therapy-resistant and hypoxia-inducing factors (HIFs) play a central role in hypoxic adaptation. Especially, renal cell carcinoma (RCC) is often associated with von Hippel-Lindau (VHL) gene mutations, leading to up-regulation of HIFs. However, from a different point of view, this suggests the possibility that HIFs could be promising targets in anti-cancer therapy. In this study, we searched for HIF-1α-derived peptides that are able to induce RCC-reactive cytotoxic T lymphocytes (CTLs) from HLA-A24(+) RCC patients...
January 19, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28103578/prognostic-and-predictive-value-of-vhl-gene-alteration-in-renal-cell-carcinoma-a-meta-analysis-and-review
#13
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 articles including following terms in their titles, abstracts, or keywords: 'kidney or renal', 'carcinoma or cancer or neoplasm or malignancy', 'von Hippel-Lindau or VHL', 'alteration or mutation or methylation', and 'prognostic or predictive'...
January 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/28099933/loss-of-maternal-chromosome-11-is-a-signature-event-in-sdhaf2-sdhd-and-vhl-related-paragangliomas-but-less-significant-in-sdhb-related-paragangliomas
#14
Attje S Hoekstra, Erik F Hensen, Ekaterina S Jordanova, Esther Korpershoek, Anouk Na van der Horst-Schrivers, Cees Cornelisse, Eleonora Pm Corssmit, Frederik J Hes, Jeroen C Jansen, Henricus Pm Kunst, Henri Jlm Timmers, Adrian Bateman, Diana Eccles, Judith Vmg Bovée, Peter Devilee, Jean-Pierre Bayley
Germline mutations in the succinate dehydrogenase (SDHA, SDHB, SDHC, SDHD, SDHAF2) or Von Hippel-Lindau (VHL) genes cause hereditary paraganglioma/pheochromocytoma. While SDHB (1p36) and VHL (3p25) are associated with autosomal dominant disease, SDHD (11q23) and SDHAF2 (11q13) show a remarkable parent-of-origin effect whereby tumor formation is almost completely dependent on paternal transmission of the mutant allele. Loss of the entire maternal copy of chromosome 11 occurs frequently in SDHD-linked tumors, and has been suggested to be the basis for this typical inheritance pattern...
January 14, 2017: Oncotarget
https://www.readbyqxmd.com/read/28096985/radioactive-seed-localization-of-renal-cell-carcinoma-in-a-patient-with-von-hippel-lindau-disease
#15
Christina Marie Schiøttz Hassing, Tove Filtenborg Tvedskov, Niels Kroman, Thomas Levin Klausen, Sissal Djurhuus, Linnea Langhans
This report describes the case of a patient, who had successful radioactive seed localization (RSL) performed to improve the identification and excision of a renal cell carcinoma. RSL is a new method of preoperative localization, which can ease the surgical procedure, minimize tissue trauma, and ultimately benefit the patient.
January 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28094316/somatostatin-receptor-expression-on-von-hippel-lindau-associated-hemangioblastomas-offers-novel-therapeutic-target
#16
Saman Sizdahkhani, Michael J Feldman, Martin G Piazza, Alexander Ksendzovsky, Nancy A Edwards, Abhik Ray-Chaudhury, Dragan Maric, Marsha J Merrill, Karel Pacak, Zhengping Zhuang, Prashant Chittiboina
Von Hippel-Lindau (VHL)-associated hemangioblastomas (VHL-HB) arise in the central nervous system (CNS), and are a leading cause of morbidity and mortality in VHL disease. Currently, surgical resection is the most effective way to manage symptomatic VHL-HBs. Surgically unresectable VHL-HBs or those in frail patients are challenging problems. Therapies targeting oncologic and vascular endothelial growth factor (VEGF) pathways have failed to demonstrate tumor control. Our experience and previous reports on VHL-HB avidity to somatostatin analogues suggested somatostatin receptor (SSTR) expression in VHL-HBs, offering an alternative therapeutic strategy...
January 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28089820/deregulated-expression-of-vhl-mrna-variants-in-papillary-thyroid-cancer
#17
Enke Baldini, Chiara Tuccilli, Yannick Arlot-Bonnemains, Frank Chesnel, Salvatore Sorrenti, Corrado De Vito, Antonio Catania, Eleonora D'Armiento, Alessandro Antonelli, Poupak Fallahi, Sara Watutantrige-Fernando, Francesco Tartaglia, Susi Barollo, Caterina Mian, Marco Bononi, Stefano Arceri, Domenico Mascagni, Massimo Vergine, Daniele Pironi, Massimo Monti, Angelo Filippini, Salvatore Ulisse
Recent findings demonstrated that a subset of papillary thyroid cancers (PTCs) is characterized by reduced expression of the von Hippel-Lindau (VHL) tumor suppressor gene, and that lowest levels associated with more aggressive PTCs. In the present study, the levels of the two VHL mRNA splicing variants, VHL-213 (V1) and VHL-172 (V2), were measured in a series of 96 PTC and corresponding normal matched tissues by means of quantitative RT-PCR. Variations in the mRNA levels were correlated with patients' clinicopathological parameters and disease-free interval (DFI)...
January 12, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28089774/rootletin-prevents-cep68-from-vhl-mediated-proteasomal-degradation-to-maintain-centrosome-cohesion
#18
Huilong Yin, Lu Zheng, Weixiao Liu, Dachuan Zhang, Wei Li, Li Yuan
Centrosome cohesion, mostly regarded as a proteinaceous linker between parental centrioles, ensures the interphase centrosome(s) to function as a single microtubule-organizing center. Maintenance of centrosome cohesion counts on a number of centrosomal linker proteins because depletion of any of those leads to premature centrosome separation in interphase, termed centrosome splitting. However, the underlying mechanisms of the dependence are unknown. Here, we show that absence of Rootletin triggers the von Hippel-Lindau tumour suppressor protein (VHL)-mediated proteasomal degradation of Cep68 and, in turn, results in centrosome splitting...
January 13, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28087080/-retinal-capillary-hemangioblastoma-in-von-hippel-lindau-disease
#19
F Benotmane, M Ez-Zahraoui, O Lezrek, O Charhi, I Ben Dali, M Laghmari, R Daoudi
No abstract text is available yet for this article.
January 10, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/28072736/von-hippel-lindau-disease-involving-pancreas-and-biliary-system-a-rare-case-report
#20
Xu-Ting Zhi, Qi-Yu Bo, Feng Zhao, Dong Sun, Tao Li
RATIONALE: Von Hippel-Lindau (VHL) disease is a rare inherited, autosomal-dominant syndrome caused by heterozygous germline mutations in the VHL gene. VHL patients are prone to develop benign and malignant tumors and cysts in multiple organ systems involving kidneys, pancreas and central nervous system (CNS). The varied and complex clinical manifestations and radiological findings of VHL are of interest. PATIENT CONCERNS: We report a 38-year-old woman with a ten-year history of VHL disease involving both pancreas and biliary system...
January 2017: Medicine (Baltimore)
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