Myelin proteolipid protein

Myelin proteolipid protein (PLP or lipophilin)
Identifiers
Symbol	Myelin_PLP
Pfam	PF01275
InterPro	IPR001614
SMART	SM00002
PROSITE	PDOC00497
Available protein structures: Pfam structures PDB RCSB PDB; PDBe PDBsum structure summary

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

GPM6A; GPM6B; PLP1;

References

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. 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 includes text from the public domain Pfam and InterPro IPR001614

This membrane protein-related article is a stub. You can help Wikipedia by expanding it.v · Categories: Protein domains Protein families Transmembrane proteins Membrane protein stubs Wikimedia Foundation. 2010. Игры ⚽ Поможем сделать НИР Myeik Myelin sheath gap Look at other dictionaries: Myelin basic protein — Rendering of MBP from PDB 1BX2 …   Wikipedia Proteolipid protein 1 — is a protein associated with Pelizaeus Merzbacher disease. It is a 4 transmembrane domain protein which binds strongly to other copies of itself on the extracellular side of the membrane. In a myelin sheath, as the layers of myelin wraps come… …   Wikipedia Myelin-Basisches Protein — Masse/Länge Primärstruktur 304 Aminosäuren …   Deutsch Wikipedia Myelin basic protein — Das Myelin Basische Protein abgek. MBP, engl.: myelin basic protein ist ein Protein, das eine große Bedeutung für die Myelinscheide der Nervenzellen besitzt. Im Proteinanteil des Myelins macht es etwa 40 Prozent aus. In der Forschung über die… …   Deutsch Wikipedia Proteolipid-Protein — Masse/Länge Primärstruktur 276 Aminosäuren …   Deutsch Wikipedia (myelin-proteolipid) O-palmitoyltransferase — In enzymology, a [myelin proteolipid] O palmitoyltransferase (EC number|2.3.1.100) is an enzyme that catalyzes the chemical reaction:palmitoyl CoA + [myelin proteolipid] ightleftharpoons CoA + O palmitoyl [myelin proteolipid] Thus, the two… …   Wikipedia Proteolipid protein (PLP) — The most abundant protein of the myelin sheath (the covering of the nerves) in the central nervous system (CNS). The gene that codes for PLP is on the X chromosome. Mutations in PLP cause Pelizaeus Merzbacher disease (PMD), an X linked recessive… …   Medical dictionary proteolipid protein — Highly conserved membrane protein (30 kD) in myelin. Cellular function obscure but mutations lethal eg. jimpy mouse and Pelizaeus Merzbacher disease of man …   Dictionary of molecular biology proteolipid protein — (PLP) a hydrophobic protein that is the major constituent of myelin in the central nervous system. Defective synthesis of PLP results in Pelizaeus Merzbacher disease. Called also lipophilin …   Medical dictionary Myelin protein zero — PDB rendering based on 1neu …   Wikipedia 18+ © Academic, 2000-2024 Contact us: Technical Support, Advertising Dictionaries export, created on PHP, Joomla, Drupal, WordPress, MODx. Mark and share Search through all dictionaries Translate… Search Internet Share the article and excerpts Direct link … Do a right-click on the link above and select “Copy Link”