Myelin proteolipid protein

Myelin proteolipid protein (PLP or lipophilin)^[1] is the major myelin protein from the central nervous system (CNS). It plays an important role in the formation or maintenance of the multilamellar structure of myelin. The myelin sheath is a multi-layered membrane, unique to the nervous system, that functions as an insulator to greatly increase the efficiency of axonal impulse conduction.^[2]

In humans, point mutations in PLP are the cause of Pelizaeus–Merzbacher disease (PMD), a neurologic disorder of myelin metabolism. In animals demyelinating diseases such as mouse 'jimpy' or dog 'shaking pup' are also caused by mutations in PLP.

PLP is a highly conserved^[3] hydrophobic protein of 276 to 280 amino acids which seems to contain four transmembrane segments, two disulfide bonds and which covalently binds lipids (at least six palmitate groups in mammals).^[4] PLP is highly related to GPM6A, a neuronal membrane glycoprotein.^[5]

GPM6A; GPM6B; PLP1;

• PLP2