Myelin proteolipid protein

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Myelin proteolipid protein (PLP or lipophilin)
Identifiers
Symbol Myelin_PLP
Pfam PF01275
InterPro IPR001614
SMART SM00002
PROSITE PDOC00497

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 velocity of axonal impulse conduction.[2]

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[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]

Human proteins containing this domain[edit]

GPM6A; GPM6B; PLP1;

References[edit]

  1. ^ 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. 
  2. ^ 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. 
  3. ^ 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. 
  4. ^ 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. 
  5. ^ 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. 

This article incorporates text from the public domain Pfam and InterPro IPR001614