Loss of von Hippel-Lindau (VHL) protein function results in an autosomal-dominant cancer syndrome known as VHL disease, which manifests as angiomas of the retina, hemangioblastomas of the central nervous system, renal clear-cell carcinomas and pheochromocytomas. VHL tumor suppressor is a specific substrate-recognition component of the E3 ubiquitin complex, which regulates proteasomal degradation of the subunit of the hypoxia inducible transcription factor (HIF). Impaired VHL complex function leads to accumulation of HIF, overexpression of various HIF-induced gene products and formation of highly vascular neoplasia. However, the ubiquitylating role of the VHL complex extends beyond its function in regulating HIF, as it appears to regulate the stability of other proteins that might be involved in various steps of oncogenic processes.

中文翻译：

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von Hippel-Lindau抑癌药：不仅是HIF的s子手。

von Hippel-Lindau（VHL）蛋白功能丧失会导致常染色体显性癌症综合征，称为VHL疾病，表现为视网膜血管瘤，中枢神经系统血管母细胞瘤，肾透明细胞癌和嗜铬细胞瘤。VHL肿瘤抑制物是E3泛素复合物的特定底物识别组件，可调节缺氧诱导转录因子（HIF）亚基的蛋白酶体降解。VHL复合功能受损会导致HIF的积累，各种HIF诱导的基因产物的过表达和高度血管瘤的形成。但是，VHL复合物的泛素化作用超出了其调控HIF的功能，因为它似乎可以调控可能与致癌过程各个步骤有关的其他蛋白质的稳定性。