Given that a high (5 mM - suprapharmacological (Wang et al., 2019)) Metformin concentration, an indirect AMPK activator, was previously reported to reduce VLCFA levels in ALD cells (Singh et al., 2016), we sought to compare the effects of PXL770 on C26:0 levels in AMN fibroblasts at different concentrations (0.1, 0.5, 1, 2, 3.5 and 5 μM) to Metformin at clinically relevant levels (LaMoia and Shulman, 2021) (100, 200, 300, and 400 μM). Here, PRKAA1 is linked to X-linked adrenoleukodystrophy.