MPZ (myelin protein zero), also known as P0, a transmembrane glycoprotein (~30 kDa), is a member of the immunoglobulin supergene family. Synthesized by myelin-forming Schwann cells, MPZ is the major structural protein component of myelin in the peripheral nervous system. It is involved in formation and maintenance of compact myelin, and plays a role in the creation of an extracellular membrane face which guides the wrapping process and ultimately compacts adjacent lamellae. More than 120 mutations detected in the gene of MPZ cause various forms of hereditary neuropathy, which include Charcot-Marie-Tooth disease type 1B (CMT1B), CMT2, Dejerine-Sottas syndrome (DSS), and congenital hypomyelination neuropathy (CHN). This antibody can recognize endogenous MPZ, and can be used as a marker of myelinating Schwann cells.
