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MeSH Review

Myelin Sheath

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Disease relevance of Myelin Sheath


Psychiatry related information on Myelin Sheath

  • Spongiform vacuoles, widespread myelin and axonal pathology, accompanied by abundant macrophagic reaction and neuroaxonal dystrophy, were consistently found in mice infected with CJD virus [6].
  • An inverse relationship between Id1/3 and myelin P0 expression was consistent with a role for these two Id proteins as inhibitors of differentiation, and Id1/3 proteins strongly repressed myelin gene promoter activity [7].
  • These significant differences in MOBP81 and MOBP170 protein kinesis coupled to different expression profiles of their respective message populations may be indicative of both myelin structural and cellular/regulatory functions, respectively, for these polypeptides [8].
  • The activity of 2',3'-cyclic nucleotide-3'-phosphodiesterase (CNPase) was determined as an index of myelin in the post-mortem brain samples of 16 patients with Alzheimer's disease (AD) and of 14 controls [9].
  • The first patient, a 4-year-old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy: post-mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis [10].

High impact information on Myelin Sheath

  • Recently we have found that unmanipulated SJL mice that are highly susceptible to EAE also maintain a very high frequency of T cells responding to an encephalitogenic epitope of a myelin antigen proteolipid protein (PLP) 139-151 in the peripheral repertoire [11].
  • Because of the advances in the understanding and treatment of EAE, recent research in MS has been focused on the characterization of cellular immune responses against myelin components [12].
  • After secretion, SP-B plays an essential role in determining the structure of tubular myelin, the stability and rapidity of spreading, and the recycling of surfactant phospholipids [13].
  • CNS myelin and sertoli cell tight junction strands are absent in Osp/claudin-11 null mice [14].
  • We hypothesized that Egr2 may be a transcription factor affecting late myelin genes and that human myelinopathies of the PNS may result from mutations in EGR2 [15].

Chemical compound and disease context of Myelin Sheath


Biological context of Myelin Sheath


Anatomical context of Myelin Sheath


Associations of Myelin Sheath with chemical compounds

  • We have used the techniques of differential screening and hybrid selection to identify a cDNA clone encoding the Schwann cell glycoprotein P0, the major structural protein of the peripheral myelin sheath [30].
  • These results indicate that GalC and sulfatide play important roles in myelin function and stability [31].
  • Deletion of the serine 34 codon from the major peripheral myelin protein P0 gene in Charcot-Marie-Tooth disease type 1B [32].
  • The mutations, glutamate substitution for lysine 96 or aspartate 90, are located in the extracellular domain, which plays a significant role in myelin membrane adhesion [33].
  • A single amino-terminal methionine is removed post-translationally, indicating that PLP does not require a signal peptide sequence for insertion into the myelin membrane [34].

Gene context of Myelin Sheath

  • As the deleted DNA region includes the coding region for a peripheral myelin gene (PMP22), we used single strand conformation analysis to examine this gene for mutations in the non-deleted HNPP family [35].
  • We conclude that Pmp22 is required for the correct development of peripheral nerves, the maintenance of axons and the determination of myelin thickness and stability [36].
  • In addition to myelin-associated glycoprotein (MAG) and Nogo-A, available evidence suggests the existence of additional inhibitors in CNS myelin [37].
  • We have generated a model of CMT-1A by introducing extra copies of the responsible disease gene, Pmp22 (encoding the peripheral myelin protein of 22 kDa), into transgenic rats [38].
  • We show here that a glycosylphosphatidylinositol (GPI)-anchored CNS myelin protein, oligodendrocyte-myelin glycoprotein (OMgp), is a potent inhibitor of neurite outgrowth in cultured neurons [37].

Analytical, diagnostic and therapeutic context of Myelin Sheath

  • In a 1-year double-blind study, 30 individuals with relapsing-remitting MS received daily capsules of bovine myelin or a control protein to determine the effect of oral tolerization to myelin antigens on the disease [39].
  • Immunohistochemical analyses of human brain sections and primary murine brain cell cultures demonstrated that TAL is expressed selectively in oligodendrocytes at high levels, possibly linked to production of large amounts of lipids as a major component of myelin, and to the protection of the vast network of myelin sheaths from oxygen radicals [40].
  • The new data show that NMDARs exist on the myelin sheath formed by oligodendrocytes, that an uncompetitive NMDAR antagonist has successfully passed human clinical trials, and that NMDARs trigger multiple deleterious cascades to inflict cellular damage on both neurons and glia during cerebral ischemia (stroke) [41].
  • Guanethidine-induced sympathectomy resulted in a Wallerian-like pattern of myelin degeneration within 10 d [42].
  • Nonpathogenic IgGs bound recombinant mouse MOG and deglycosylated MOG in myelin (tested by Western blot), but only pathogenic IgGs bound glycosylated MOG [43].


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  2. Shaping of the autoreactive T-cell repertoire by a splice variant of self protein expressed in thymic epithelial cells. Klein, L., Klugmann, M., Nave, K.A., Tuohy, V.K., Kyewski, B. Nat. Med. (2000) [Pubmed]
  3. Uncoupling of hypomyelination and glial cell death by a mutation in the proteolipid protein gene. Schneider, A., Montague, P., Griffiths, I., Fanarraga, M., Kennedy, P., Brophy, P., Nave, K.A. Nature (1992) [Pubmed]
  4. T-cell epitope of the autoantigen myelin basic protein that induces encephalomyelitis. Zamvil, S.S., Mitchell, D.J., Moore, A.C., Kitamura, K., Steinman, L., Rothbard, J.B. Nature (1986) [Pubmed]
  5. CD62L is required on effector cells for local interactions in the CNS to cause myelin damage in experimental allergic encephalomyelitis. Grewal, I.S., Foellmer, H.G., Grewal, K.D., Wang, H., Lee, W.P., Tumas, D., Janeway, C.A., Flavell, R.A. Immunity (2001) [Pubmed]
  6. Reevaluation of the ultrastructural pathology of experimental Creutzfeldt-Jakob disease. Serial studies of the Fujisaki strain of Creutzfeldt-Jakob disease virus in mice. Liberski, P.P., Yanagihara, R., Asher, D.M., Gibbs, C.J., Gajdusek, D.C. Brain (1990) [Pubmed]
  7. Reciprocal Id expression and myelin gene regulation in Schwann cells. Thatikunta, P., Qin, W., Christy, B.A., Tennekoon, G.I., Rutkowski, J.L. Mol. Cell. Neurosci. (1999) [Pubmed]
  8. Cytoskeletal and nuclear localization of myelin oligodendrocytic basic protein isoforms. Montague, P., Barrie, J.A., Thomson, C.E., Kirkham, D., McCallion, A.S., Davies, R.W., Kennedy, P.G., Griffths, I.R. Eur. J. Neurosci. (1998) [Pubmed]
  9. 2',3'-cyclic nucleotide-3'-phosphodiesterase activity as an index of myelin in the post-mortem brains of patients with Alzheimer's disease. Reinikainen, K.J., Pitkänen, A., Riekkinen, P.J. Neurosci. Lett. (1989) [Pubmed]
  10. Infantile and juvenile presentations of Alexander's disease: a report of two cases. Deprez, M., D'Hooghe, M., Misson, J.P., de Leval, L., Ceuterick, C., Reznik, M., Martin, J.J., D'Hooge, M. Acta neurologica Scandinavica. (1999) [Pubmed]
  11. T cell response in experimental autoimmune encephalomyelitis (EAE): role of self and cross-reactive antigens in shaping, tuning, and regulating the autopathogenic T cell repertoire. Kuchroo, V.K., Anderson, A.C., Waldner, H., Munder, M., Bettelli, E., Nicholson, L.B. Annu. Rev. Immunol. (2002) [Pubmed]
  12. Immunological aspects of demyelinating diseases. Martin, R., McFarland, H.F., McFarlin, D.E. Annu. Rev. Immunol. (1992) [Pubmed]
  13. Human surfactant protein B: structure, function, regulation, and genetic disease. Whitsett, J.A., Nogee, L.M., Weaver, T.E., Horowitz, A.D. Physiol. Rev. (1995) [Pubmed]
  14. CNS myelin and sertoli cell tight junction strands are absent in Osp/claudin-11 null mice. Gow, A., Southwood, C.M., Li, J.S., Pariali, M., Riordan, G.P., Brodie, S.E., Danias, J., Bronstein, J.M., Kachar, B., Lazzarini, R.A. Cell (1999) [Pubmed]
  15. Mutations in the early growth response 2 (EGR2) gene are associated with hereditary myelinopathies. Warner, L.E., Mancias, P., Butler, I.J., McDonald, C.M., Keppen, L., Koob, K.G., Lupski, J.R. Nat. Genet. (1998) [Pubmed]
  16. Neonatal hypothyroidism affects the timely expression of myelin-associated glycoprotein in the rat brain. Rodriguez-Peña, A., Ibarrola, N., Iñiguez, M.A., Muñoz, A., Bernal, J. J. Clin. Invest. (1993) [Pubmed]
  17. Membrane-type 1 matrix metalloprotease (MT1-MMP) enables invasive migration of glioma cells in central nervous system white matter. Beliën, A.T., Paganetti, P.A., Schwab, M.E. J. Cell Biol. (1999) [Pubmed]
  18. Vaccination for protection of retinal ganglion cells against death from glutamate cytotoxicity and ocular hypertension: implications for glaucoma. Schori, H., Kipnis, J., Yoles, E., WoldeMussie, E., Ruiz, G., Wheeler, L.A., Schwartz, M. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  19. Childhood peripheral neuropathy with autoantibodies to myelin glycoprotein P0. Ben Jelloun-Dellagi, S., Dellagi, K., Burger, D., Ben Younes-Chennoufi, A., Hentati, F.F., Steck, A., Ben Hamida, M. Ann. Neurol. (1992) [Pubmed]
  20. Trembler mouse carries a point mutation in a myelin gene. Suter, U., Welcher, A.A., Ozcelik, T., Snipes, G.J., Kosaras, B., Francke, U., Billings-Gagliardi, S., Sidman, R.L., Shooter, E.M. Nature (1992) [Pubmed]
  21. TNF alpha promotes proliferation of oligodendrocyte progenitors and remyelination. Arnett, H.A., Mason, J., Marino, M., Suzuki, K., Matsushima, G.K., Ting, J.P. Nat. Neurosci. (2001) [Pubmed]
  22. B lymphocytes producing demyelinating autoantibodies: development and function in gene-targeted transgenic mice. Litzenburger, T., Fässler, R., Bauer, J., Lassmann, H., Linington, C., Wekerle, H., Iglesias, A. J. Exp. Med. (1998) [Pubmed]
  23. Tyrosine phosphorylation of QKI mediates developmental signals to regulate mRNA metabolism. Zhang, Y., Lu, Z., Ku, L., Chen, Y., Wang, H., Feng, Y. EMBO J. (2003) [Pubmed]
  24. Oligodendroglial modulation of fast axonal transport in a mouse model of hereditary spastic paraplegia. Edgar, J.M., McLaughlin, M., Yool, D., Zhang, S.C., Fowler, J.H., Montague, P., Barrie, J.A., McCulloch, M.C., Duncan, I.D., Garbern, J., Nave, K.A., Griffiths, I.R. J. Cell Biol. (2004) [Pubmed]
  25. Complement-mediated demyelination in patients with IgM monoclonal gammopathy and polyneuropathy. Monaco, S., Bonetti, B., Ferrari, S., Moretto, G., Nardelli, E., Tedesco, F., Mollnes, T.E., Nobile-Orazio, E., Manfredini, E., Bonazzi, L. N. Engl. J. Med. (1990) [Pubmed]
  26. Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons. Giese, K.P., Martini, R., Lemke, G., Soriano, P., Schachner, M. Cell (1992) [Pubmed]
  27. Identification of autoantibodies associated with myelin damage in multiple sclerosis. Genain, C.P., Cannella, B., Hauser, S.L., Raine, C.S. Nat. Med. (1999) [Pubmed]
  28. The structural gene coding for myelin-associated proteolipid protein is mutated in jimpy mice. Dautigny, A., Mattei, M.G., Morello, D., Alliel, P.M., Pham-Dinh, D., Amar, L., Arnaud, D., Simon, D., Mattei, J.F., Guenet, J.L. Nature (1986) [Pubmed]
  29. Peripheral nerve grafts in hereditary leukodystrophic mutant mice (twitcher). Scaravilli, F., Jacobs, J.M. Nature (1981) [Pubmed]
  30. Isolation and sequence of a cDNA encoding the major structural protein of peripheral myelin. Lemke, G., Axel, R. Cell (1985) [Pubmed]
  31. Myelination in the absence of galactocerebroside and sulfatide: normal structure with abnormal function and regional instability. Coetzee, T., Fujita, N., Dupree, J., Shi, R., Blight, A., Suzuki, K., Suzuki, K., Popko, B. Cell (1996) [Pubmed]
  32. Deletion of the serine 34 codon from the major peripheral myelin protein P0 gene in Charcot-Marie-Tooth disease type 1B. Kulkens, T., Bolhuis, P.A., Wolterman, R.A., Kemp, S., te Nijenhuis, S., Valentijn, L.J., Hensels, G.W., Jennekens, F.G., de Visser, M., Hoogendijk, J.E. Nat. Genet. (1993) [Pubmed]
  33. Charcot-Marie-Tooth neuropathy type 1B is associated with mutations of the myelin P0 gene. Hayasaka, K., Himoro, M., Sato, W., Takada, G., Uyemura, K., Shimizu, N., Bird, T.D., Conneally, P.M., Chance, P.F. Nat. Genet. (1993) [Pubmed]
  34. Nucleotide sequences of two mRNAs for rat brain myelin proteolipid protein. Milner, R.J., Lai, C., Nave, K.A., Lenoir, D., Ogata, J., Sutcliffe, J.G. Cell (1985) [Pubmed]
  35. A frame shift mutation in the PMP22 gene in hereditary neuropathy with liability to pressure palsies. Nicholson, G.A., Valentijn, L.J., Cherryson, A.K., Kennerson, M.L., Bragg, T.L., DeKroon, R.M., Ross, D.A., Pollard, J.D., McLeod, J.G., Bolhuis, P.A. Nat. Genet. (1994) [Pubmed]
  36. Hypermyelination and demyelinating peripheral neuropathy in Pmp22-deficient mice. Adlkofer, K., Martini, R., Aguzzi, A., Zielasek, J., Toyka, K.V., Suter, U. Nat. Genet. (1995) [Pubmed]
  37. Oligodendrocyte-myelin glycoprotein is a Nogo receptor ligand that inhibits neurite outgrowth. Wang, K.C., Koprivica, V., Kim, J.A., Sivasankaran, R., Guo, Y., Neve, R.L., He, Z. Nature (2002) [Pubmed]
  38. Therapeutic administration of progesterone antagonist in a model of Charcot-Marie-Tooth disease (CMT-1A). Sereda, M.W., Meyer zu Hörste, G., Suter, U., Uzma, N., Nave, K.A. Nat. Med. (2003) [Pubmed]
  39. Double-blind pilot trial of oral tolerization with myelin antigens in multiple sclerosis. Weiner, H.L., Mackin, G.A., Matsui, M., Orav, E.J., Khoury, S.J., Dawson, D.M., Hafler, D.A. Science (1993) [Pubmed]
  40. Oligodendrocyte-specific expression and autoantigenicity of transaldolase in multiple sclerosis. Banki, K., Colombo, E., Sia, F., Halladay, D., Mattson, D.H., Tatum, A.H., Massa, P.T., Phillips, P.E., Perl, A. J. Exp. Med. (1994) [Pubmed]
  41. NMDA receptors, glial cells, and clinical medicine. Lipton, S.A. Neuron (2006) [Pubmed]
  42. Myelin sheath survival after guanethidine-induced axonal degeneration. Kidd, G.J., Heath, J.W., Trapp, B.D., Dunkley, P.R. J. Cell Biol. (1992) [Pubmed]
  43. Pathogenic myelin oligodendrocyte glycoprotein antibodies recognize glycosylated epitopes and perturb oligodendrocyte physiology. Marta, C.B., Oliver, A.R., Sweet, R.A., Pfeiffer, S.E., Ruddle, N.H. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
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