Peripheral myelin protein 2 (PMP2)

Peripheral myelin protein 2 (PMP2) is a small β-barrel fatty acid-binding protein localized predominantly to the myelin sheaths of the peripheral nervous system[1][2][3]. It binds monomeric lipids (fatty acids, cholesterol) inside its cavity, and its hydrophobic, positively charged exterior interacts directly with membranes and promotes their stacking, contributing crucially to the formation and maintenance of compact myelin structure needed for proper nerve insulation and conduction[1][2][3]. Mutations in the PMP2 gene have been causally linked to dominant forms of demyelinating Charcot–Marie–Tooth peripheral neuropathy, typically resulting in destabilized protein, impaired lipid binding, and abnormal myelin ultrastructure manifesting as reduced nerve conduction and clinical neuropathy symptoms[1][2][3]. Recent research further shows PMP2 can influence sphingomyelin organization at the plasma membrane in a PI(4,5)P2-dependent manner and may be regulated by SOX10, implicating it in melanoma cell invasion[1]. Thus, PMP2 is an essential lipid-binding and structural protein of peripheral myelin, with established roles both in neurological disease and possibly cancer biology.

Other names

Myelin P2 proteinPMP2MP2FABP8M-FABPP2CMT1G