PRKAA1 and X-linked adrenoleukodystrophy: In ALD patient-derived cells, metformin - a weak and indirect AMPK activator - at a suprapharmacological concentration (5mM - (Wang et al., 2019)) was shown to decrease levels of VLCFA, improve mitochondrial function, reduce markers of inflammation and dose-dependently induce ABCD2 expression in ALD lymphocytes (Singh et al., 2016).