Dystrophin Related Protein 2 (DRP2)

During the course of a phylogenetic study of sequences encoding the characteristic C-terminal domains of dystrophin-related proteins, Roberts et al. (1996) identified a vertebrate dystrophin-related gene class. They termed this class dystrophin-related protein-2 (DRP2) and suggested that utrophin/DRP be designated DRP1 to simplify future nomenclature.

DRP2 gene is expressed principally in the brain and spinal cord.rodent Drp2 interacted and colocalized with periaxin (PRX) within a dystroglycan complex localized to the plasma membrane of Schwann cells. In Prx-null mice, who develop a severe late-onset demyelinating neuropathy in which the myelin sheath becomes unstable. periaxin-Drp2-dystroglycan complex is important in regulating the terminal stages of myelination.