Deletion of various negative HIF regulators, the HIF inhibitor (FIH/HIF1AN) or the von Hippel–Lindau disease tumour suppressor (VHL) protein, or the prolyl hydroxylase domain-containing proteins (PHD), or overexpression of modified, negative regulator-insensitive HIF-ɑ proteins, exacerbates the terminal effector program (Clever et al., 2016; Doedens et al., 2013; Finlay et al., 2012; Liikanen et al., 2021; Palazon et al., 2017; Veliça et al., 2021). This evidence concerns the gene VHL and glycogen storage disease VI.