In early pathological investigations of PXE, Iliás et al. in 2002 proposed that ABCC6 is a transporter of organic anions and actively transports glutathione conjugates, including leukotriene C4 and N-ethylmaleimide S-glutathione (NEM-GS), and their abolishment due to missense mutation in ABCC6 gene is responsible for PXE [26,27]. The gene discussed is ABCC6; the disease is Pseudoxanthoma elasticum.