Our biochemical studies, which include enzyme kinetics and cellular localization data, along with the studies from others (5) suggest speculatively that given ABHD12's preference for VLC lipids, it functions as a major lipase controlling the concentrations and flux of the VLC lipids (lyso-PS), which are constantly biosynthesized in the ER membrane, and perturbation in the activity of ABHD12 causes unchecked accumulation of these VLC lipids particularly lyso-PS lipids, resulting in the pathophysiology observed in PHARC subjects. Here, ABHD12 is linked to PHARC syndrome.