The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

PLP1  -  proteolipid protein 1

Homo sapiens

Synonyms: GPM6C, HLD1, Lipophilin, MMPL, Myelin proteolipid protein, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of PLP1


Psychiatry related information on PLP1

  • This report extends the phenotypic range of disease due to PLP gene mutations to include adult-onset dementia in females [6].
  • We found robust activations in cortical motor areas [areas 4a, 4p, 6; dorsal premotor cortex (PMD) and bilateral supplementary motor area (SMA)] and ipsilateral cerebellum during kinesthetic illusions (illusion-vibration) [7].
  • These studies have focused primarily on schizophrenia, and less so on the affective psychoses, including psychotic major depression PMD [8].
  • We tested whether periodic maternal deprivation (180 min daily on postnatal days 3-10, PMD) caused chronic changes in emotional behavior and HPA axis activity in either male or female adult rats, or both [9].
  • We have proposed a three-class hierarchical specificity model for sub-typing the depressive disorders (comprising psychotic, melancholic and non-melancholic depression), with specificity referring to two clinical features (psychotic symptoms and psychomotor disturbance or PMD) separating the first two classes from a residual non-melancholic class [10].

High impact information on PLP1


Chemical compound and disease context of PLP1


Biological context of PLP1


Anatomical context of PLP1


Associations of PLP1 with chemical compounds

  • These data demonstrate that expression of PLP1 but not DMSO is necessary to prevent neuropathy, and suggest that the 35 amino acid PLP1-specific domain plays an important role in normal peripheral nerve function [3].
  • When myelin or white matter was extracted with chloroform/methanol, it contained, in addition to PLP, a significant amount of plasmolipin [23].
  • Lipid analysis revealed that the PLP-MBP-S-MAG coimmunoprecipitated with some phospholipids and sulfatide but not cholesterol or galactosylceramide [24].
  • The PLP gene in one of two affected males and the carrier mother of this family exhibited a single base difference in the more than 2 kb of the PLP gene sequenced, a C----T transition that would create a serine substitution for proline at the carboxy end of the protein [25].
  • A single T----C transition results in the substitution of a charged amino acid residue, arginine, for tryptophan in one of the four extremely hydrophobic domains of the PLP protein [26].

Physical interactions of PLP1


Regulatory relationships of PLP1

  • Thus, one means whereby zinc stabilises association of MBP with brain myelin membranes may be by promoting its binding to PLP [28].
  • We also found that glucocorticoids stimulate the translation of MBP and PLP mRNAs and inhibit the translation of CNP mRNA in cell-free systems [29].
  • Collectively, these results indicate that myelin proteolipid protein peptide-specific CD8+ CTL may be an important source of proinflammatory soluble mediators that could promote and mediate the inflammatory response in MS demyelination [30].

Other interactions of PLP1

  • An immunohistochemical study, which can detect the specific expression of PLP, myelin basic protein, myelin-associated glycoprotein, and platelet-derived growth factor receptor alpha subunit in brain tissues, showed that the myelination was almost the same as that of age-matched controls [31].
  • PLP and MAG were detected rostrally between 12 to 14 GW [32].
  • The replicase polyprotein of murine coronavirus is extensively processed by three proteinases, two papain-like proteinases (PLPs), termed PLP1 and PLP2, and a picornavirus 3C-like proteinase (3CLpro) [33].
  • The differential expression seen by microarray for CART as well as MBP, MOBP, and PLP was verified by RT-PCR [34].
  • We found that there was higher frequency of increased T-cell reactivity to immunodominant PLP peptides in women than in men, particularly in non-MS individuals [35].

Analytical, diagnostic and therapeutic context of PLP1


  1. Heterogeneous duplications in patients with Pelizaeus-Merzbacher disease suggest a mechanism of coupled homologous and nonhomologous recombination. Woodward, K.J., Cundall, M., Sperle, K., Sistermans, E.A., Ross, M., Howell, G., Gribble, S.M., Burford, D.C., Carter, N.P., Hobson, D.L., Garbern, J.Y., Kamholz, J., Heng, H., Hodes, M.E., Malcolm, S., Hobson, G.M. Am. J. Hum. Genet. (2005) [Pubmed]
  2. Spastic paraplegia type 2 associated with axonal neuropathy and apparent PLP1 position effect. Lee, J.A., Madrid, R.E., Sperle, K., Ritterson, C.M., Hobson, G.M., Garbern, J., Lupski, J.R., Inoue, K. Ann. Neurol. (2006) [Pubmed]
  3. Schwann cell expression of PLP1 but not DM20 is necessary to prevent neuropathy. Shy, M.E., Hobson, G., Jain, M., Boespflug-Tanguy, O., Garbern, J., Sperle, K., Li, W., Gow, A., Rodriguez, D., Bertini, E., Mancias, P., Krajewski, K., Lewis, R., Kamholz, J. Ann. Neurol. (2003) [Pubmed]
  4. Primary progressive multiple sclerosis as a phenotype of a PLP1 gene mutation. Warshawsky, I., Rudick, R.A., Staugaitis, S.M., Natowicz, M.R. Ann. Neurol. (2005) [Pubmed]
  5. Identification of mouse hepatitis virus papain-like proteinase 2 activity. Kanjanahaluethai, A., Baker, S.C. J. Virol. (2000) [Pubmed]
  6. Adult-onset neurodegenerative disorder due to proteolipid protein gene mutation in the mother of a man with Pelizaeus-Merzbacher disease. Nance, M.A., Boyadjiev, S., Pratt, V.M., Taylor, S., Hodes, M.E., Dlouhy, S.R. Neurology (1996) [Pubmed]
  7. Dominance of the right hemisphere and role of area 2 in human kinesthesia. Naito, E., Roland, P.E., Grefkes, C., Choi, H.J., Eickhoff, S., Geyer, S., Zilles, K., Ehrsson, H.H. J. Neurophysiol. (2005) [Pubmed]
  8. Neuropsychological correlates of psychotic features in major depressive disorders: a review and meta-analysis. Fleming, S.K., Blasey, C., Schatzberg, A.F. Journal of psychiatric research. (2004) [Pubmed]
  9. Periodic maternal deprivation induces gender-dependent alterations in behavioral and neuroendocrine responses to emotional stress in adult rats. Wigger, A., Neumann, I.D. Physiol. Behav. (1999) [Pubmed]
  10. Modelling late-life depression. Parker, G., Snowdon, J., Parker, K. International journal of geriatric psychiatry. (2003) [Pubmed]
  11. A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease. Gow, A., Lazzarini, R.A. Nat. Genet. (1996) [Pubmed]
  12. Mice lacking tissue non-specific alkaline phosphatase die from seizures due to defective metabolism of vitamin B-6. Waymire, K.G., Mahuren, J.D., Jaje, J.M., Guilarte, T.R., Coburn, S.P., MacGregor, G.R. Nat. Genet. (1995) [Pubmed]
  13. X-linked spastic paraplegia and Pelizaeus-Merzbacher disease are allelic disorders at the proteolipid protein locus. Saugier-Veber, P., Munnich, A., Bonneau, D., Rozet, J.M., Le Merrer, M., Gil, R., Boespflug-Tanguy, O. Nat. Genet. (1994) [Pubmed]
  14. Pelizaeus-Merzbacher disease: a valine to phenylalanine point mutation in a putative extracellular loop of myelin proteolipid. Pham-Dinh, D., Popot, J.L., Boespflug-Tanguy, O., Landrieu, P., Deleuze, J.F., Boué, J., Jollès, P., Dautigny, A. Proc. Natl. Acad. Sci. U.S.A. (1991) [Pubmed]
  15. Brain N-acetylaspartate is elevated in Pelizaeus-Merzbacher disease with PLP1 duplication. Takanashi, J., Inoue, K., Tomita, M., Kurihara, A., Morita, F., Ikehira, H., Tanada, S., Yoshitome, E., Kohno, Y. Neurology (2002) [Pubmed]
  16. Combination of systemic hypothermia and N-acetylcysteine attenuates hypoxic-ischemic brain injury in neonatal rats. Jatana, M., Singh, I., Singh, A.K., Jenkins, D. Pediatr. Res. (2006) [Pubmed]
  17. Abnormal regulation of plasma pyridoxal 5'-phosphate in patients with liver disease. Mitchell, D., Wagner, C., Stone, W.J., Wilkinson, G.R., Schenker, S. Gastroenterology (1976) [Pubmed]
  18. Induction of the myelin proteolipid protein (PLP) gene in C6 glioblastoma cells: functional analysis of the PLP promotor. Nave, K.A., Lemke, G. J. Neurosci. (1991) [Pubmed]
  19. Genomic rearrangements resulting in PLP1 deletion occur by nonhomologous end joining and cause different dysmyelinating phenotypes in males and females. Inoue, K., Osaka, H., Thurston, V.C., Clarke, J.T., Yoneyama, A., Rosenbarker, L., Bird, T.D., Hodes, M.E., Shaffer, L.G., Lupski, J.R. Am. J. Hum. Genet. (2002) [Pubmed]
  20. Mild Pelizaeus-Merzbacher disease caused by a point mutation affecting correct splicing of PLP1 mRNA. Hübner, C.A., Senning, A., Orth, U., Zerres, K., Urbach, H., Gal, A., Rudnik-Schöneborn, S. Neuroscience (2005) [Pubmed]
  21. The pathobiology of myelin mutants reveal novel biological functions of the MBP and PLP genes. Campagnoni, A.T., Skoff, R.P. Brain Pathol. (2001) [Pubmed]
  22. Surges of increased T cell reactivity to an encephalitogenic region of myelin proteolipid protein occur more often in patients with multiple sclerosis than in healthy subjects. Pender, M.P., Csurhes, P.A., Greer, J.M., Mowat, P.D., Henderson, R.D., Cameron, K.D., Purdie, D.M., McCombe, P.A., Good, M.F. J. Immunol. (2000) [Pubmed]
  23. Presence of the plasma membrane proteolipid (plasmolipin) in myelin. Cochary, E.F., Bizzozero, O.A., Sapirstein, V.S., Nolan, C.E., Fischer, I. J. Neurochem. (1990) [Pubmed]
  24. Myelin proteolipid protein, basic protein, the small isoform of myelin-associated glycoprotein, and p42MAPK are associated in the Triton X-100 extract of central nervous system myelin. Arvanitis, D.N., Yang, W., Boggs, J.M. J. Neurosci. Res. (2002) [Pubmed]
  25. Pelizaeus-Merzbacher disease: an X-linked neurologic disorder of myelin metabolism with a novel mutation in the gene encoding proteolipid protein. Gencic, S., Abuelo, D., Ambler, M., Hudson, L.D. Am. J. Hum. Genet. (1989) [Pubmed]
  26. Mutation of the proteolipid protein gene PLP in a human X chromosome-linked myelin disorder. Hudson, L.D., Puckett, C., Berndt, J., Chan, J., Gencic, S. Proc. Natl. Acad. Sci. U.S.A. (1989) [Pubmed]
  27. Purification and characterization of the mammaglobin/lipophilin B complex, a promising diagnostic marker for breast cancer. Carter, D., Douglass, J.F., Cornellison, C.D., Retter, M.W., Johnson, J.C., Bennington, A.A., Fleming, T.P., Reed, S.G., Houghton, R.L., Diamond, D.L., Vedvick, T.S. Biochemistry (2002) [Pubmed]
  28. Zinc ions stabilise the association of basic protein with brain myelin membranes. Earl, C., Chantry, A., Mohammad, N., Glynn, P. J. Neurochem. (1988) [Pubmed]
  29. Posttranscriptional events in the expression of myelin protein genes. Campagnoni, A.T., Verdi, J.M., Verity, A.N., Amur-Umarjee, S. Ann. N. Y. Acad. Sci. (1990) [Pubmed]
  30. Chemokine and matrix metalloproteinase secretion by myelin proteolipid protein-specific CD8+ T cells: potential roles in inflammation. Biddison, W.E., Taub, D.D., Cruikshank, W.W., Center, D.M., Connor, E.W., Honma, K. J. Immunol. (1997) [Pubmed]
  31. Myelination of a fetus with Pelizaeus-Merzbacher disease: immunopathological study. Shiraishi, K., Itoh, M., Sano, K., Takashima, S., Kubota, T. Ann. Neurol. (2003) [Pubmed]
  32. Temporal and spatial expression of major myelin proteins in the human fetal spinal cord during the second trimester. Weidenheim, K.M., Bodhireddy, S.R., Rashbaum, W.K., Lyman, W.D. J. Neuropathol. Exp. Neurol. (1996) [Pubmed]
  33. Identification of the murine coronavirus MP1 cleavage site recognized by papain-like proteinase 2. Kanjanahaluethai, A., Jukneliene, D., Baker, S.C. J. Virol. (2003) [Pubmed]
  34. Gene expression profile of the nucleus accumbens of human cocaine abusers: evidence for dysregulation of myelin. Albertson, D.N., Pruetz, B., Schmidt, C.J., Kuhn, D.M., Kapatos, G., Bannon, M.J. J. Neurochem. (2004) [Pubmed]
  35. Effect of gender on T-cell proliferative responses to myelin proteolipid protein antigens in patients with multiple sclerosis and controls. Greer, J.M., Csurhes, P.A., Pender, M.P., McCombe, P.A. J. Autoimmun. (2004) [Pubmed]
  36. PLP1-related inherited dysmyelinating disorders: Pelizaeus-Merzbacher disease and spastic paraplegia type 2. Inoue, K. Neurogenetics (2005) [Pubmed]
  37. Expression of myelin proteins in the developing human spinal cord: cloning and sequencing of human proteolipid protein cDNA. Kronquist, K.E., Crandall, B.F., Macklin, W.B., Campagnoni, A.T. J. Neurosci. Res. (1987) [Pubmed]
  38. Expression of recombinant forms of human 21.5 kDa myelin basic protein and proteolipid protein in CHO cells. Jaśkiewicz, E., Jedynak, A., Zioło, E. Acta Biochim. Pol. (2005) [Pubmed]
  39. Pelizaeus-Merzbacher disease: detection of mutations Thr181----Pro and Leu223----Pro in the proteolipid protein gene, and prenatal diagnosis. Strautnieks, S., Rutland, P., Winter, R.M., Baraitser, M., Malcolm, S. Am. J. Hum. Genet. (1992) [Pubmed]
  40. Duplication of the proteolipid protein gene is the major cause of Pelizaeus-Merzbacher disease. Sistermans, E.A., de Coo, R.F., De Wijs, I.J., Van Oost, B.A. Neurology (1998) [Pubmed]
WikiGenes - Universities