PROSITE documentation PDOC00497
Myelin proteolipid protein signatures


Myelin proteolipid protein (PLP or lipophilin) [1] is the major myelin protein from the central nervous system (CNS). It probably plays an important role in the formation or maintenance of the multilamellar structure of myelin. 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' (jp), rat md, or dog 'shaking pup' are also caused by mutations in PLP.

PLP is a highly conserved [2] 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) [3].

PLP is highly related to M6, a neuronal membrane glycoprotein [4].

As signature patterns for these proteins, we selected two conserved regions. The first pattern is located in the N-terminal region; at the extremity of the first transmembrane region. The second pattern is located in the extracellular loop between the last two transmembrane regions and contains a cysteine involved in a disulfide bond.

Expert(s) to contact by email:

Hofmann K.

Last update:

July 1999 / Text revised.


Technical section

PROSITE methods (with tools and information) covered by this documentation:

MYELIN_PLP_1, PS00575; Myelin proteolipid protein signature 1  (PATTERN)

MYELIN_PLP_2, PS01004; Myelin proteolipid protein signature 2  (PATTERN)


1AuthorsPopot J.-L. Pham Dinh D. Dautigny A.
TitleMajor Myelin proteolipid: the 4-alpha-helix topology.
SourceJ. Membr. Biol. 120:233-246(1991).
PubMed ID1711121

2AuthorsSchliess F. Stoffel W.
TitleEvolution of the myelin integral membrane proteins of the central nervous system.
SourceBiol. Chem. Hoppe-Seyler 372:865-874(1991).
PubMed ID1722981

3AuthorsWeimbs T. Stoffel W.
TitleProteolipid 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.
SourceBiochemistry 31:12289-12296(1992).
PubMed ID1281423

4AuthorsYan Y. Lagenaur C. Narayanan V.
TitleMolecular cloning of M6: identification of a PLP/DM20 gene family.
SourceNeuron 11:423-431(1993).
PubMed ID8398137

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