hemangioblastoma/hypoxia

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Accelerated growth of hemangioblastoma in pregnancy: the role of proangiogenic factors and upregulation of hypoxia-inducible factor (HIF) in a non-oxygen-dependent pathway.

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Logáil Isteach / Cláraigh

Hemangioblastomas (HBs) are benign, highly vascular tumors, often characterized by loss of function of the von Hippel-Lindau (vHL) gene. They are the most common central nervous system tumor observed in vHL syndrome. Loss of function of the vHL gene creates a "pseudo-hypoxic" state, causing

Hypoxia mediated expression of stem cell markers in VHL-associated hemangioblastomas.

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Logáil Isteach / Cláraigh

Hemangioblastomas of the retina, central nervous system, and kidney are observed in patients with mutations in the von Hippel-Lindau (VHL) tumor suppressor gene. Mutations in the VHL lead to constitutive activation of hypoxia-inducible-factor (HIF) pathway. HIF-mediated expression of pro-angiogenic

Expression of HIF-1 regulated proteins vascular endothelial growth factor, carbonic anhydrase IX and hypoxia inducible gene 2 in hemangioblastomas.

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Logáil Isteach / Cláraigh

BACKGROUND Hemangioblastomas occur as sporadic or as von Hippel-Lindau syndrome (VHL) associated tumors. In both settings, activation of the VHL-HIF-1 (hypoxia induced factor) pathway is thought to be important in tumor biology. METHODS We performed immunohistochemical studies on 23

Upregulation of hypoxia-inducible factors and autophagy in von Hippel-Lindau-associated retinal hemangioblastoma.

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Logáil Isteach / Cláraigh

OBJECTIVE To describe pathological and molecular changes of three patients with clinically severe von Hippel-Lindau (VHL)-associated retinal hemangioblastoma (RH) with rapid progression. METHODS Medical records, ocular histopathology, and transmission electron microscopy from three cases of

[Reactive erythrocythemia without recognizable hypoxemia. Isolated erythrocythemia in Lindau's angioblastoma].

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Logáil Isteach / Cláraigh

Deletion of the von Hippel-Lindau Gene in Hemangioblasts Causes Hemangioblastoma-like Lesions in Murine Retina.

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Logáil Isteach / Cláraigh

von Hippel-Lindau (VHL) disease is an autosomal-dominant tumor predisposition syndrome characterized by the development of highly vascularized tumors and cysts. LOH of the VHL gene results in aberrant upregulation of hypoxia-inducible factors (HIF) and has been associated with tumor formation.

Emetine promotes von Hippel-Lindau-independent degradation of hypoxia-inducible factor-2α in clear cell renal carcinoma.

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Logáil Isteach / Cláraigh

Inactivating mutations of the von Hippel-Lindau (VHL) tumor suppressor gene are associated with inherited VHL syndrome, which is characterized by susceptibility to a variety of neoplasms, including central nervous system hemangioblastoma and clear cell renal cell carcinoma (CCRCC). Mutations in the

Soft-tissue hemangioblastoma of the retroperitoneum: a case study and review of the literature.

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Logáil Isteach / Cláraigh

A 71-year-old woman was found to have a solid retroperitoneal mass measuring 7 x 6 cm on a computed tomography scan. The tumor was not connected to the spinal cord or large nerves. It was resected and showed the typical histology of hemangioblastoma, that is, numerous capillaries and stromal cells

Endolymphatic sac tumors in patients with and without von Hippel-Lindau disease: the role of genetic mutation, von Hippel-Lindau protein, and hypoxia inducible factor-1alpha expression.

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Logáil Isteach / Cláraigh

OBJECTIVE Endolymphatic sac (ELS) tumors are low-grade malignancies of the temporal bone that are associated with von Hippel-Lindau (VHL) disease but can also occur sporadically. The VHL gene product VHL protein is important in the regulation of hypoxia inducible factor (HIF)-1alpha, which controls

Molecular pathology and CXCR4 expression in surgically excised retinal hemangioblastomas associated with von Hippel-Lindau disease.

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Logáil Isteach / Cláraigh

OBJECTIVE The surgical excision of retinal vascular lesions including hemangioblastomas is rarely practiced. This study investigates the pathological characteristics of 4 patients (3 with von Hippel-Lindau [VHL] disease and 1 with a vasoproliferative retinal tumor) who underwent ocular tumor

Regulation of STRA13 by the von Hippel-Lindau tumor suppressor protein, hypoxia, and the UBC9/ubiquitin proteasome degradation pathway.

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Logáil Isteach / Cláraigh

In this study, we focus on different modes of regulation of STRA13, a human ortholog of the mouse basic helix-loop-helix transcriptional factor, previously identified by us as a new von Hippel-Lindau tumor suppressor gene (VHL) target. The gene was overexpressed in VHL-deficient cell lines and

Expression of angiopoietins in renal epithelial and clear cell carcinoma cells: regulation by hypoxia and participation in angiogenesis.

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Logáil Isteach / Cláraigh

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

Clusterin is a secreted marker for a hypoxia-inducible factor-independent function of the von Hippel-Lindau tumor suppressor protein.

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Logáil Isteach / Cláraigh

Germline mutations in the von Hippel-Lindau (VHL) tumor suppressor gene predispose people to renal cancer, hemangioblastomas, and pheochromocytomas in an allele-specific manner. The best documented function of the VHL gene product (pVHL) relates to its ability to polyubiquitinate, and hence target

Loss of heterozygosity reveals non-VHL allelic loss in hemangioblastomas at 22q13.

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Logáil Isteach / Cláraigh

Hemangioblastomas (HBs) are low-grade (World Health Organization grade I/IV) central nervous system (CNS) tumors that frequently contain VHL (3p26) mutations. They occur sporadically and in von Hippel Lindau (VHL) disease. Encoded pVHL aids degradation of hypoxia-inducible factors (HIFs) in the

[Cerebellar hemangioblastomas--a study of the immunoprofile of neoplastic stromal component].

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Logáil Isteach / Cláraigh

BACKGROUND Central nervous system hemangioblastomas (HBs) are uncommon, highly vascularized tumors that are predominantly found in the cerebellum. They occur sporadically or in association with von Hippel-Lindau (VHL) disease. HBs are of unknown histogenesis, and the origin of stromal cells is still

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