Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 20 of 30 for “"Von Hippel-Lindau"”.
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The Role of Von Hippel-Lindau Protein in the Glomerulus
… In this thesis, we show that deletion of the Von Hippel-Lindau gene (Vhlh) from intrinsic glomerular cells of mice is sufficient to initiate a necrotizing crescentic glomerulonephritis and the clinical features that accompany RPGN. Loss of Vhlh leads to stabilization of hypoxia-inducible …
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Hämangioblastome des Zentralen Nervensystems und die von Hippel-Lindau Krankheit
… Manifestation der autosomal dominant vererbten von Hippel-Lindau Krankheit <br>auf, die sich des weiteren im Wesentlichen durch retinale Angiome, <br>Nierenzellkarzinome und Phäochromozytome auszeichnet. Um die Bedeutung einer genetischen Untersuchung konstitutioneller DNA für Patienten mit …
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Morphogenesis of follicular epithelium in Drosophila melanogaster: function of von Hippel-Lindau tumour suppressor gene
… function of the Drosophila homolog of the human von Hippel-Lindau (d-VHL) during oogenesis, in order to gain insight into the role of h-VHL for the pathogenesis of VHL disease. h-VHL is implicated in a variety of processes and there is now a greater appreciation of HIF-independent h-VHL functions …
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Von Hippel-Lindau Syndrome: Characterization of a Potentially Novel VEGF-A Isoform and Elucidation of Molecular and Vascular Mechanisms of Observed Phenotypic Changes
Von Hippel-Lindau (VHL) syndrome is an autosomal dominant predisposition to cancer in neurological tissues, the kidneys, adrenal glands, pancreas, and liver, including neurological hemangioblastoma (HB), pheochromocytoma (PCC), pancreatic neuroendocrine tumors (PNET), pancreatic and renal cysts, …
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Die Von-Hippel-Lindau-Deletion im proximalen Tubulus schützt die Niere vor der Entwicklung der diabetischen Nephropathie im Frühstadium des Diabetes mellitus Typ 1
… mellitus Typ 1 und 2 und die häufigste Ursache von Dialysepflichtigkeit. Das Frühstadium ist durch eine glomeruläre Hyperfiltration und Albuminurie gekennzeichnet. Dadurch ist der proximale Tubulus einem erhöhten glukosereichen Filtrat ausgesetzt. Er ist der metabolisch aktivste Teil der Niere …
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Identification de microARNs impliqués dans le développement des carcinomes rénaux
… l'inactivation du gène de suppression tumoral von Hippel-Lindau (VHL) qui est observé dans plus de 80% des cas. Une meilleure compréhension de ces tumeurs permettra d'améliorer la détection et le développement des thérapies ciblées pour les ccRCCs. Les microARNs (miARNs) sont des petits …
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Modulation du métabolisme des sphingolipides avec une petite molécule, le STF-62247, dans le but de cibler les carcinomes rénaux
… l'inactivation du gène supresseur de tumeurs, von Hippel-Lindau (VHL). Des études préalables ont permis d'identifier une petite molécule, le STF-62247, ayant la capacité de cibler spécifiquement les cellules déficientes en VHL. Cette sélectivité module le processus autophagique et des …
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Effet d'une petite molécule sur les transporteurs d'acides aminés pour cibler les tumeurs déficientes en VHL par l'autophagie
… une inactivation du gène de supression tumoral von Hippel-Lindau (VHL). Dans la poursuite de nouvelles thérapies ciblées, des études antérieures ont permis d'identifier une petite molécule, le STF-62247, qui est capable de cibler cette inactivation du VHL. Alors que son mécanisme d'action …
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Protein structure and interaction under environmental stress : from quality control recognition to evolution of collective behavior
… susceptible to misfolding is the human von Hippel-Lindau (VHL) tumor suppressor. When expressed in the absence of its cofactors, VHL cannot fold correctly and is quickly degraded by the cell's quality control machinery. Here, I present a biophysical characterization of a VHL mutation …
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Notch Signaling in Neuroblastoma and Renal Cell Carcinoma
… (HIF) pathway due to functional loss of the von Hippel-Lindau tumor suppressor gene. However, cooperating oncogenic events parallel to the HIF pathway remains poorly described. Our results presented here imply a growth-promoting role for Notch signaling in CCRCC cells, seemingly independent …
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The regulation of hypoxia-responsive gene expression by hydroxyl radicals and intracellular calcium
… Die Fenton-Reaktion konnte durch den Gebrauch von nicht-fluoreszierendem Dihydrorhodamin (DHR) lokalisiert werden, das durch die in der Fenton Reaktion entstehenden OH· zum fluoreszierenden Rhodamin (RH) umgesetzt wird. Diese Fluoreszenz kann dann zusammen mit der Fluoreszenz von …
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Fragment-based approaches to probe the surface of the VHL E3 ligase
… this work, interactions of components of the von Hippel-Lindau (VHL) E3 ligase were investigated using small-molecules. By applying an integrated set of biophysical and structural biology techniques, the main goals of this project were to provide chemical tools and novel assays to probe …
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VHL inhibitors as chemical probes of the hypoxia signalling pathway
Von Hippel–Lindau (VHL) is the E3 ubiquitin ligase targeting hypoxia-inducible transcription factor-alpha (HIF α) for proteasomal degradation. The crucial function of VHL in response to hypoxia and cellular oxygen sensing are well established, owing to the use of genetic tools through knockout and …
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Understanding patient engagement: exploring factors around utilisation of screening and surveillance services in individuals with or at risk of rare endocrine syndromes
… with rare genetic endocrine syndromes, such as von-Hippel Lindau, Multiple Endocrine Neoplasia and mutations in the succinate dehydrogenase complex genes can develop diverse and unpredictable new tumour formations. Regular screening is recommended as best practice for early detection and …
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Μελέτη των μοριακών μηχανισμών ανάπτυξης του οφθαλμικού πτερυγίου στον άνθρωπο : ο ρόλος του μεταγραφικού παράγοντα HIF-1α, της πρωτεΐνης pVHL και των πρωτεΐνών θερμικού σοκ
… Σε φυσιολογικές συνθήκες οξυγόνου η πρωτεΐνη von Hippel Lindau (pVHL) διατηρεί τα επίπεδα της δραστικότητας του HIF-1α χαμηλά. Επιπλέον, πολλά είναι τα ερευνητικά δεδομένα που αποκαλύπτουν τη συνεργασία των οδών ρύθμισης των επιπέδων του οξυγόνου και της έκφρασης των πρωτεϊνών θερμικού σοκ …
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Novel Proteolysis Targeting Chimeras for the targeted degradation of the protein kinase CK2
… a CAM4066-derived warhead to Cereblon (CRBN) and Von Hippel–Lindau (VHL) E3-ligase ligands via a linker. The project was split into three stages: development of a synthetic route towards PROTACs; synthesis of PROTAC candidates, and evaluation of their biological activity in vitro. Four VHL-based …
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Erythropoietin Signaling in Pancreatic Beta Cells in Homeostasis and in Models of Type 1 and Type 2 Diabetes
… (HIF) pathway, we hypothesized that deletion of von Hippel Lindau (VHL), a negative regulator of this pathway, in the β cells would lead to enhanced transcription of HIF-target genes, which are largely pro-survival, and lead to enhanced β-cell mass and function. Contrary to our hypothesis, in …
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AMBIENT OXYGEN AVAILABILITY MODULATES EXPRESSION OF VASCULAR ANGIOGENIC FACTORS AND CAPILLARY REMODELING (ANGIOPLASTICITY) IN THE MOUSE BRAIN
… but there was no change in HIF-1¿¿ mRNA and von Hippel Lindau (VHL) protein expression during hyperoxia, with capillary density significantly diminished by the 3rd week of hyperoxia. In the third part of the study, we analyzed whether aging affects systemic and cerebral angiogenic adaptive …
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Cibler la vulnérabilité lysosomale dans le cancer du rein héréditaire comme nouvelle approche thérapeutique
La maladie Von Hippel-Lindau (VHL) est un syndrome héréditaire causé par des mutations sur le gène VHL entraînant le développement de tumeurs dans différents organes tels que l’œil, l’oreille, les glandes surrénales, le cerveau, le pancréas et les reins. Environ 70% des patients atteints de la …
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Endogenous Reporter Systems for High-Throughput Functional Screening in Clear Cell Renal Cell Carcinoma
… clear cell renal cell carcinoma (ccRCC), loss of von Hippel-Lindau tumour suppressor (VHL), seen in 90% of ccRCCs, leads to the stabilization of hypoxia inducible factor HIF2A and aberrant expression of its downstream targets such as the chemokine receptor CXCR4. Activation of HIF2A has been shown …
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