proteolipid protein


Gene Symbol: proteolipid protein
Description: proteolipid protein 1
Alias: Plp, myelin proteolipid protein, Proteolipid protein (Pelizaeus-Merzbacher disease, spastic paraplegia 2, uncomplicated), lipophilin, proteolipid protein (myelin) 1
Species: rat
Products:     proteolipid protein

Top Publications

  1. Bayless D, Daniel J. Sex differences in myelin-associated protein levels within and density of projections between the orbital frontal cortex and dorsal striatum of adult rats: implications for inhibitory control. Neuroscience. 2015;300:286-96 pubmed publisher
    ..In Experiment 1, we measured levels of two myelin-associated proteins, myelin basic protein (MBP) and myelin proteolipid protein (PLP), in the OFC and dSTR...
  2. Wang E, Huang Z, Hobson G, Dimova N, Sperle K, McCullough A, et al. PLP1 alternative splicing in differentiating oligodendrocytes: characterization of an exonic splicing enhancer. J Cell Biochem. 2006;97:999-1016 pubmed
    b>Proteolipid protein (PLP) and DM20 are generated by alternative splicing of exon 3B of PLP1 transcript in differentiating oligodendrocytes...
  3. Yamaguchi Y, Miyagi Y, Baba H. Two-dimensional electrophoresis with cationic detergents: a powerful tool for the proteomic analysis of myelin proteins. Part 2: analytical aspects. J Neurosci Res. 2008;86:766-75 pubmed
  4. Boison D, Stoffel W. Myelin-deficient rat: a point mutation in exon III (A----C, Thr75----Pro) of the myelin proteolipid protein causes dysmyelination and oligodendrocyte death. EMBO J. 1989;8:3295-302 pubmed
    The expression of the proteolipid protein (PLP) gene of the myelin deficient (md) and normal rat was studied during the myelination period. The sizes of the PLP transcripts (1.6 and 3...
  5. Dhaunchak A, Nave K. A common mechanism of PLP/DM20 misfolding causes cysteine-mediated endoplasmic reticulum retention in oligodendrocytes and Pelizaeus-Merzbacher disease. Proc Natl Acad Sci U S A. 2007;104:17813-8 pubmed
    ..Here we show that a major subgroup of PMD mutations that map into the extracellular loop region of PLP/DM20 leads to the failure of oligodendrocytes to form the correct intramolecular disulfide bridges...
  6. De Monasterio Schrader P, Patzig J, Mobius W, Barrette B, Wagner T, Kusch K, et al. Uncoupling of neuroinflammation from axonal degeneration in mice lacking the myelin protein tetraspanin-2. Glia. 2013;61:1832-47 pubmed publisher
    Deficiency of the major constituent of central nervous system (CNS) myelin, proteolipid protein (PLP), causes axonal pathology in spastic paraplegia type-2 patients and in Plp1(null) -mice but is compatible with almost normal myelination...
  7. Dautigny A, Alliel P, d Auriol L, Pham Dinh D, Nussbaum J, Galibert F, et al. Molecular cloning and nucleotide sequence of a cDNA clone coding for rat brain myelin proteolipid. FEBS Lett. 1985;188:33-6 pubmed pBR322 and screened with a 14-mer mixed oligonucleotide probe based on residues 231-235 of bovine proteolipid (PLP)...
  8. Simons R, Riordan J. Single base substitution in codon 74 of the MD rat myelin proteolipid protein gene. Ann N Y Acad Sci. 1990;605:146-54 pubmed
    ..It appears in all to be the result of mutations in the myelin proteolipid protein gene which is located on the long arm of the X-chromosome...
  9. Griffiths I, Scott I, McCulloch M, Barrie J, McPhilemy K, Cattanach B. Rumpshaker mouse: a new X-linked mutation affecting myelination: evidence for a defect in PLP expression. J Neurocytol. 1990;19:273-83 pubmed
    ..Immunostaining demonstrates a major defect in expression of PLP DM-20...

More Information


  1. Padhi B, Rosales M, Pelletier G. Perinatal methylmercury exposure perturbs the expression of Plp1 and Cnp splice variants in cerebellum of rat pups. Neurotoxicology. 2015;48:223-30 pubmed publisher
    ..The Proteolipid Protein 1 (Plp1), Myelin Basic Protein (Mbp) and 2',3'-Cyclic Nucleotide 3'Phosphodiesterase (Cnp) genes expressed ..
  2. Amiguet P, Gardinier M, Zanetta J, Matthieu J. Purification and partial structural and functional characterization of mouse myelin/oligodendrocyte glycoprotein. J Neurochem. 1992;58:1676-82 pubmed
    ..Reduced MOG concentrations were observed in jimpy and quaking dysmyelinating mutant mice, giving further support to its localization in compact myelin of the CNS. ..
  3. Noetzli L, Sanz P, Brodsky G, Hinckley J, Giugni J, Giannaula R, et al. A novel mutation in PLP1 causes severe hereditary spastic paraplegia type 2. Gene. 2014;533:447-50 pubmed publisher
    Hereditary spastic paraplegia (HSP) type 2 is a proteolipid protein (PLP1)-related genetic disorder that is characterized by dysmyelination of the central nervous system resulting primarily in limb spasticity, cognitive impairment, ..
  4. Mayer C, Macklin W, Avishai N, Balan K, Wilson C, Miller M. Mutation in the myelin proteolipid protein gene alters BK and SK channel function in the caudal medulla. Respir Physiol Neurobiol. 2009;169:303-14 pubmed publisher
    b>Proteolipid protein (Plp) gene mutation in rodents causes severe CNS dysmyelination, early death, and lethal hypoxic ventilatory depression (Miller et al., 2004)...
  5. Ozgen H, Schrimpf W, Hendrix J, de Jonge J, Lamb D, Hoekstra D, et al. The lateral membrane organization and dynamics of myelin proteins PLP and MBP are dictated by distinct galactolipids and the extracellular matrix. PLoS ONE. 2014;9:e101834 pubmed publisher
    ..5-kDa myelin basic protein (MBP) and transmembrane protein proteolipid protein (PLP) as a function of the typical myelin lipids galactosylceramide (GalC), and sulfatide, and exogenous ..
  6. Murad S, Kishimoto Y. Alpha hydroxylation of lignoceric acid to cerebronic acid during brain development. Diminished hydroxylase activity in myelin-deficient mouse mutants. J Biol Chem. 1975;250:5841-6 pubmed
    ..These results suggest a close association of the synthesis of cerebronic acid with the synthesis of the characteristic myelin lipid that is cerebroside (N-acyl sphingosine beta-D-galactoside)...
  7. Hudson L. Pelizaeus-Merzbacher disease and spastic paraplegia type 2: two faces of myelin loss from mutations in the same gene. J Child Neurol. 2003;18:616-24 pubmed
    ..Both arise from mutations in the gene encoding myelin proteolipid protein. The disease spectrum for Pelizaeus-Merzbacher disease and spastic paraplegia type 2 is extraordinarily ..
    ..Both mutants offer new opportunities for study of the formation and functions of myelin. ..
  9. Gudz T, Schneider T, Haas T, Macklin W. Myelin proteolipid protein forms a complex with integrins and may participate in integrin receptor signaling in oligodendrocytes. J Neurosci. 2002;22:7398-407 pubmed
    ..A new role for the myelin proteolipid protein (PLP), the most abundant protein of CNS myelin, has been identified, in studies showing PLP interaction ..
  10. Boucher S, Cypher M, Carlock L, Skoff R. Proteolipid protein gene modulates viability and phenotype of neurons. J Neurosci. 2002;22:1772-83 pubmed
    Overexpression or lack of expression of proteolipid protein (PLP) gene by oligodendrocytes causes axonal pathology...
  11. Yamamoto T, Nanba E. A novel mutation (A246T) in exon 6 of the proteolipid protein gene associated with connatal Pelizaeus-Merzbacher disease. Hum Mutat. 1999;14:182 pubmed
  12. Nave K, Lai C, Bloom F, Milner R. Splice site selection in the proteolipid protein (PLP) gene transcript and primary structure of the DM-20 protein of central nervous system myelin. Proc Natl Acad Sci U S A. 1987;84:5665-9 pubmed
    b>Proteolipid protein (PLP) is the major myelin membrane protein of the central nervous system...
  13. Schaich M, Budzinski R, Stoffel W. Cloned proteolipid protein and myelin basic protein cDNA. Transcription of the two genes during myelination. Biol Chem Hoppe Seyler. 1986;367:825-34 pubmed
    cDNA clones of rat brain proteolipid protein (PLP), also named lipophilin, the major integral myelin membrane protein, and of myelin basic protein (MBP), the major extrinsic myelin protein, have been isolated from a rat brain cDNA ..
  14. Milner R, Lai C, Nave K, Lenoir D, Ogata J, Sutcliffe J. Nucleotide sequences of two mRNAs for rat brain myelin proteolipid protein. Cell. 1985;42:931-9 pubmed
    The 3200 and 1600 nucleotide mRNAs encoding rat brain proteolipid protein (PLP), the major protein component of central nervous system myelin, are heterogeneous at their 5' ends, differ in their 3' polyadenylation sites, and are ..
  15. Csiza C, de Lahunta A. Myelin deficiency (md): a neurologic mutant in the Wistar rat. Am J Pathol. 1979;95:215-24 pubmed
    ..The only gross postmortem change is a gray color of the spinal cord instead of its normally white appearance. Microscopic findings reveal total lack of myelin formation at all levels of the central nervous system. ..
  16. Spörkel O, Uschkureit T, Bussow H, Stoffel W. Oligodendrocytes expressing exclusively the DM20 isoform of the proteolipid protein gene: myelination and development. Glia. 2002;37:19-30 pubmed
    ..of the main myelin proteins, the peripheral myelin basic isoproteins (MBP) and the integral proteolipid proteins, PLP and DM20, arise from alternative splicing...
  17. Nadon N, Duncan I. Gene expression and oligodendrocyte development in the myelin deficient rat. J Neurosci Res. 1995;41:96-104 pubmed
    ..The myelin deficient (md) rat, carrying a Thr75-->Pro substitution present in both isoforms of proteolipid protein (PLP and DM20), is the most severely affected of the PLP mutants described to date...
  18. Duncan I, Hammang J, Trapp B. Abnormal compact myelin in the myelin-deficient rat: absence of proteolipid protein correlates with a defect in the intraperiod line. Proc Natl Acad Sci U S A. 1987;84:6287-91 pubmed
    ..fixation methods showed that the myelinated fibers were positive for myelin basic protein but negative for proteolipid protein (PLP)...
  19. Gardinier M, Macklin W, Diniak A, Deininger P. Characterization of myelin proteolipid mRNAs in normal and jimpy mice. Mol Cell Biol. 1986;6:3755-62 pubmed
    A clone specific for the rat myelin proteolipid protein (PLP) was isolated from a cDNA library made in pUC18 from 17-day-old rat brain stem mRNA. This clone corresponded to the carboxyl-terminal third of the PLP-coding region...
  20. Parenti R, Cicirata F, Zappala A, Catania A, La Delia F, Cicirata V, et al. Dynamic expression of Cx47 in mouse brain development and in the cuprizone model of myelin plasticity. Glia. 2010;58:1594-609 pubmed publisher
    ..Taken as a whole the evidence suggests that Cx47 play a key role in myelination. ..
  21. Mayer J, Larsen E, Kondo Y, Duncan I. Characterization of a PLP-overexpressing transgenic rat, a model for the connatal form of Pelizaeus-Merzbacher disease. Neurobiol Dis. 2011;44:231-8 pubmed publisher
    ..Altered expression of Plp1 also caused an increase in the apoptotic cell death of oligodendrocytes. These results provide the platform from which to test the effectiveness of in vivo therapies. ..
  22. Simons R, Riordan J. The myelin-deficient rat has a single base substitution in the third exon of the myelin proteolipid protein gene. J Neurochem. 1990;54:1079-81 pubmed
    ..Some of these disorders are inherited in an X-linked manner. The localization of the myelin proteolipid protein (PLP) gene to the X chromosome has directed the study of X-linked myelination disorders toward PLP...
  23. Baron W, Ozgen H, Klunder B, de Jonge J, Nomden A, Plat A, et al. The major myelin-resident protein PLP is transported to myelin membranes via a transcytotic mechanism: involvement of sulfatide. Mol Cell Biol. 2015;35:288-302 pubmed publisher
    ..nature of oligodendrocytes, we demonstrate that transcytotic transport of the major myelin-resident protein proteolipid protein (PLP) is a key element in the mechanism of myelin assembly...
  24. Gordon M, Kumar S, Espinosa de los Monteros A, de Vellis J. Ontogeny of glycerol phosphate dehydrogenase-positive oligodendrocytes in rat brain. Impaired differentiation of oligodendrocytes in the myelin deficient mutant rat. Int J Dev Neurosci. 1992;10:243-53 pubmed expressed relatively early in oligodendrocyte differentiation, prior to either myelin basic protein or proteolipid protein expression...
  25. Takeda K, Dezawa M, Kitada M. The expression of PLP/DM-20 mRNA is restricted to the oligodendrocyte-lineage cells in the adult rat spinal cord. Histochem Cell Biol. 2016;145:147-61 pubmed publisher
    b>Proteolipid protein (PLP) is the major component of myelin; its gene encodes two major splicing variants: PLP and DM-20. Compared with PLP, DM-20 lacks the amino acids encoded by exon IIIb...