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]
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Transcription
Human proteins containing this domain
See also
References
- ^ 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. S2CID 24450880.
- ^ 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. doi:10.1016/S0021-9258(18)61334-1. 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. S2CID 46719251.
This page was last edited on 29 November 2023, at 02:22