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CTNI-82. BELZUTIFAN TREATMENT OF VHL-ASSOCIATED CENTRAL NERVOUS SYSTEM HEMANGIOBLASTOMAdoi:10.1093/neuonc/noae165.0449Von Hippel-Lindau (VHL) disease is caused by pathogenic variants in the VHL gene. Abnormal function of VHL protein leads to accumulation of HIF-伪 and the engagement of hypoxia-...
von Hippel–Lindau disease (VHL) is a rare autosomal dominant condition that causes benign and malignant tumors and cysts in the central nervous system (CNS) as well as visceral organs. VHL results from a mutation in the VHL tumor suppressor gene on chromosome 3p25.3. One of the most common...
and possesses ubiquitin ligase E3 activity. This protein is involved in the ubiquitination and degradation of hypoxia-inducible-factor (HIF), which is a transcription factor that plays a central role in the regulation of gene expression by oxygen. RNA polymerase II subunit POLR2G/RPB7 is also re...
The identification in 1993 of inherited mutations in the von Hippel–Lindau (VHL) gene in families with VHL disease was a seminal finding. This and subsequent discoveries have given the VHLtumour suppressor gene a central role in our understanding of the
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The von Hippel–Lindau (VHL) tumor suppressor is a multisubunit Cullin RING E3 ligase (CRL2VHL)—composed of Cullin2 as the central scaffold subunit, Rbx1 as RING subunit, ElonginB and ElonginC as adaptor subunits, and VHL as substrate recognition subunit (1, 2). VHL functions to specifica...
central nervous system hemangioblastoma; RCC, renal cell carcinoma; PCT, multiple pancreatic cysts or tumors; Pheo, pheochromocytoma.C–JImaging and pathological findings of the patient III-1 of family 1.C,D: abdominal MRI showed multiple occupying lesions in the kidney;Emultiple pancreatic cysts;...
The von Hippel-Lindau (VHL) is deficient in ∼70% of clear-cell renal cell carcinomas (ccRCC), which contributes to the carcinogenesis and drug resistance of ccRCC. Here we show that VHL-deficient ccRCC cells present enhanced cytotoxicity of anthracycl
Given the central role of VHL in renal cancer formation, we examined the VHL-dependent regulation of miRNAs in renal cancer. Methods VHL-dependent miRNA expression in cc RCC was determined by microarray analysis of renal cell line RCC4 with mutated VHL (RCC4-VHL) and reintroduced wild-type ...