Myelin protein P0

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The MPZ (P0, CHM, DSS, MPP, CMT1, CMT1B, CMT2I, CMT2J, CMT4E, CMTDI3, HMSNIB) gene at 1q23.3 codes for myelin protein P0 (myelin protein zero, myelin peripheral protein, MPP) a major structural protein of peripheral myelin expressed only by Schwann cells. The function of this protein once N-glycosylated is creation of an extracellular membrane face which guides the wrapping process and ultimately compacts adjacent myelin lamellae. Abnormalities in the gene are associated with various forms of peroneal muscular atrophy and other CNS conditions including:

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