DRP2 (dystrophin related protein 2) is a component of the dystroglycan complex essential for normal myelination and Schwann cell organization in peripheral nerves 1. The protein is required for the formation of Cajal bands, which are cytoplasmic channels in myelinating Schwann cells, and for proper localization of periaxin at appositions between the myelin sheath and Schwann cell plasma membrane 1. DRP2 must interact with periaxin to form these critical appositions, and this interaction is necessary for DRP2 phosphorylation 1. Located on the X chromosome X Xq22, DRP2 is primarily expressed in brain and spinal cord tissues 2. Pathogenic variants in DRP2 cause X-linked Charcot-Marie-Tooth disease, characterized by late-onset sensory and motor neuropathy with intermediate conduction velocities in affected males, while heterozygous females remain asymptomatic 3. The absence of DRP2 leads to disrupted myelin appositions, abnormal myelin thickness, and focal hypermyelination followed by demyelination 1. The primary function of the DRP2-containing dystroglycan complex appears to be stabilizing and limiting radial myelin growth, making it crucial for proper peripheral nerve myelination 1.