Myelin protein zero (MPZ)

Myelin protein zero (MPZ) is the most abundant protein component of myelin in the peripheral nervous system (PNS), where it is produced and expressed by Schwann cells[2][3][5]. It is a type I transmembrane glycoprotein comprised of an extracellular immunoglobulin-like domain, a single transmembrane segment, and a highly positively charged cytoplasmic tail[3]. MPZ functions as both a structural and cell adhesion molecule, serving as molecular "glue" that mediates the compaction and stabilization of the myelin sheath by binding together the cytoplasmic and extracellular faces of Schwann cell membrane layers[2][3][5]. This adhesive function is essential for the formation and maintenance of mature, compact myelin, crucial for efficient nerve conduction in peripheral nerves. Failure or mutations in MPZ disrupt myelin compaction, leading to various inherited peripheral neuropathies such as Charcot–Marie–Tooth disease and Dejerine–Sottas syndrome[2][3][5]. MPZ is not a classical drug target, nor is it known to directly interact with therapeutic drugs, but its mutations are used as genetic biomarkers for diagnosis and may present future therapeutic challenges concerning myelin repair or stabilization[5][6].

Other names

Myelin protein P0P0 protein