VHL基因具有调节转录、稳定细胞生长相关基因组和调节细胞周期的功能 ,其抑癌作用主要是通过wt pVHL对转录延长因子ElonginABC复合物形成的抑制 ,从而发挥对细胞生长相关基因表达的调控作用。VHL基因突变、缺失、重排和异常甲基化在血管网织细胞瘤中有很高的检出率 ,这一事实有力地证明了VHL基因异常与血管网织细胞瘤的发生密切相关。 VHL gene has functions of regulating transcription, stabilizing cell growth-related genomes and regulating cell cycle. Its tumor suppressive effect is mainly through the inhibition of transcriptional elongation factor ElonginABC complex formation by wt pVHL, thereby exerting a regulatory effect on cell growth-related gene expression. The high detection rate of VHL gene mutations, deletions, rearrangements, and aberrant methylation in angiomyoblastic tumors strongly demonstrates that VHL gene abnormalities are closely related to the occurrence of vascular reticulonoma.