VHL基因突变与肾癌 - 知乎 - 知乎专栏
2021年8月FDA批准低氧诱导因子-2α (HIF-2α)抑制剂Welireg(belzutifan)上市，用于治疗希佩尔·林道综合征 ( von Hippel-Lindau，VHL) 疾病相关癌症，包括：肾细胞癌(RCC)、中枢神经系 …
Belzutifan for Renal Cell Carcinoma in von Hippel–Lindau Disease
2021年11月24日 · In this phase 2, open-label, single-group trial, we investigated the efficacy and safety of the HIF-2α inhibitor belzutifan (MK-6482, previously called PT2977), administered …
VHL Gene Mutations in Renal Cell Carcinoma: Role as a …
von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary disorder characterized by retinal and CNS hemangioblastomas, pheochromocytoma, and clear cell renal cell carcinoma. …
Tumor heterogeneity in VHL drives metastasis in clear cell renal …
2023年4月17日 · In this study, our VHL-deleted RCC models reveal a novel metastatic mechanism that relies on cooperative interactions between two distinct populations of tumor …
Renal Cell Carcinoma in von Hippel–Lindau Disease—From Tumor Genetics ...
Affected individuals are at increased risk of developing recurrent and bilateral kidney cysts and dysplastic lesions which may progress to clear cell renal cell carcinoma (ccRCC). Following …
Role of VHL gene mutation in human renal cell carcinoma
The VHL gene protein has multiple functions that are linked to tumor suppression, but the best recognized and evidently linked to the development of renal cell carcinoma (RCC) is inhibition …
von Hippel-Lindau mutants in renal cell carcinoma are ... - Nature
2019年3月19日 · Renal cell carcinoma (RCC) is the major cause of death among patients with von Hippel-Lindau (VHL) disease. Resistance to therapies targeting tumor angiogenesis...
HIF-1α and HIF-2α differently regulate tumour development and ...
2020年8月17日 · Mutational inactivation of VHL is the earliest genetic event in the majority of clear cell renal cell carcinomas (ccRCC), leading to accumulation of the HIF-1α and HIF-2α …
Sequential pathogenesis of metastatic VHL mutant clear cell …
2016年9月1日 · Clear cell renal cell carcinoma (ccRCC) accounts for ∼80% of all RCC and the Von Hippel–Lindau (VHL) tumor suppressor gene is mutated, deleted or epigenetically …
Metabolomic landscape of renal cell carcinoma in von Hippel-Lindau ...
2024年7月19日 · Von Hippel-Lindau (VHL) syndrome is a rare autosomal dominant disorder, where renal cell carcinoma (RCC) serves as a significant cause of mortality. We collected …