EPAS1 and von Hippel-Lindau disease: Its known genetic background (VHL syndrome) allows us to suspect the hypoxic inductivity of CSPG4. The up-regulation of the CSPG4 at 48 h but not 3 h of oxygen reduction indicates a link to chronic hypoxia, thus revealing CSPG4 as a possible target gene of HIF2a; that fits the HIF2a dependency of VHL syndrome [40], [41].