Biol. 22(15):5319-5336. Mol. Cell. Hippel-Lindau Tumor Suppressor Protein. Nuclear-Cytoplasmic Trafficking of the von Degradation of Hypoxia-Inducible Factor Requires Oxygen-Dependent Ubiquitination and 2002. Isabelle Groulx and Stephen Lee Suppressor Protein the von Hippel-Lindau Tumor Nuclear-Cytoplasmic Trafficking of Hypoxia-Inducible Factor Requires and Degradation of Oxygen-Dependent Ubiq...

OBJECTIVES Cerebellar haemangioblastoma occurs sporadically or as a component tumour of autosomal dominant von Hippel-Lindau disease. Biallelic inactivation of the VHL tumour suppressor gene, which is located on chromosome 3p, has been shown to be involved in the pathogenesis of both tumour entities. Mechanisms of VHL inactivation are intragenic mutations, mitotic recombination events, and hype...

We wish to report a cluster of kidney malignancies (adenocarcinoma) that has occurred among three young male utility workers who were responsible for maintaining electrical transmission equipment including power transformers. Each was employed by a single public utility company during the same period and worked at some time repairing and maintaining transformers. The most common coolants used i...

Von Hippel-Lindau disease is a rare, familial disease consisting of multiple tumors, which can present in the eyes, brain, adrenal gland, pancreas, liver, spinal cord, kidneys, or other areas of the body. The renal tumors are primarily renal cell carcinoma. Renal involvement may be the primary manifestation, but most frequently it is a secondary manifestation. This article presents an overview ...

Hemangioblastomas are rare vascular tumors most often found in the posterior fossa and cervical spinal cord and commonly associated with von Hippel-Lindau Disease. We report a case of sporadic hemangioblastoma in a patient without von Hippel-Lindau Disease. Imaging characteristics included a solid, suprasellar mass that was homogeneously enhancing. These findings most resembled a pituicytoma or...

A probable diagnosis of von HippelLindau disease was made in a two generation family in which the proband had a phaeochromocytoma, renal cysts, and multiple cerebral cavernomas. His sister had multiple similar cerebral vascular lesions and his father died from renal carcinoma aged 42. Although the family did not satisfy the conventional diagnostic criteria for von Hippel-Lindau disease, an unde...

We describe five patients diagnosed with von Hippel-Lindau disease who complained of abdominal distension, pain and discomfort for a long time. All patients underwent ultrasonography, CT scan and MRI, which showed huge pancreas filled with multiple cysts. Additionally, extrapancreatic findings such as cerebellar hemangioblastoma (3 patients), retinal hemangioblastoma (2), renal cell carcinoma (...

A 25-year-old male patient presented with a one-month history of progressive right visual loss. Magnetic resonance imaging showed 20x18 mm cystic lesion which has a mural nodule component with contrast and arisen from optic chiasm, optic nerve and optic tract. Right frontotemporal craniotomy was performed and tumor was totally resected. The histological diagnosis was hemangioblastoma. The patie...