Our previous findings suggested a hypothesis in which LCA may cause such remodeling of mitochondrial lipidome by 1) slowing down the Psd1-dependent reaction of the synthesis of PE from PS in the IMM and/or OMM; 2) decelerating the Crd1-dependent reaction of CL synthesis from PG in the IMM; and 3) attenuating a negative feedback loop that involves a CL-dependent inhibition of PA transport from the OMM across the IMS to the IMM, which is catalyzed by the Ups1/Mdm35 protein complex (Figure 1) [60]. Here, PSD is linked to Leber congenital amaurosis.