Meanwhile, Maynard et al.16, 17found that the HIF3A variant was downregulated in primary clear cell renal cell carcinomas and that von Hippel‐Lindau (VHL) gene mutations activated the hypoxia response pathway, and further exogenous expression of HIF‐3 α 4 variants suppressed the advancement of VHL‐null renal cell carcinoma. Here, VHL is linked to hereditary clear cell renal cell carcinoma.