Myelin proteolipid protein
|Myelin proteolipid protein (PLP or lipophilin)|
Myelin proteolipid protein (PLP or lipophilin) 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.
In man point mutations in PLP are the cause of Pelizaeus-Merzbacher disease (PMD), a neurologic disorder of myelin metabolism. In animals dismyelinating diseases such as mouse 'jimpy' or dog 'shaking pup' are also caused by mutations in PLP.
PLP is a highly conserved 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). PLP is highly related to GPM6A, a neuronal membrane glycoprotein.
Human proteins containing this domain
- Dautigny A, Popot JL, Pham Dinh D (1991). "Major Myelin proteolipid: the 4-alpha-helix topology". J. Membr. Biol. 120 (3): 233–246. doi:10.1007/BF01868534. PMID 1711121.
- Kitamura K, Sakamoto Y, Yoshimura K, Nishijima T, Uyemura K (1987). "Complete amino acid sequence of PO protein in bovine peripheral nerve myelin". J. Biol. Chem. 262 (9): 4208–4214. PMID 2435734.
- Stoffel W, Schliess F (1991). "Evolution of the myelin integral membrane proteins of the central nervous system". Biol. Chem. Hoppe-Seyler. 372 (9): 865–874. doi:10.1515/bchm3.1991.372.2.865. PMID 1722981.
- Weimbs T, Sto ffel W (1992). "Proteolipid protein (PLP) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues and implications for the membrane topology of PLP". Biochemistry. 31 (49): 12289–12296. doi:10.1021/bi00164a002. PMID 1281423.
- Yan Y, Lagenaur C, Narayanan V (1993). "Molecular cloning of M6: identification of a PLP/DM20 gene family". Neuron. 11 (3): 423–431. doi:10.1016/0896-6273(93)90147-J. PMID 8398137.