In this study, we demonstrated that CG11077, an MPV17/PMP22 family gene, is a Drosophila ortholog of mammalian Mpv17 and named it dMpv17. Human Mpv17 is known as a causal gene of MPV17-related hepatocerebral MDDS and CMT4,6,8, and models of these diseases have been developed in several organisms such as the mouse and zebrafish10,17. Here, PMP22 is linked to Charcot-Marie-Tooth disease type 4.