Skip to main content
SpringerLink
Log in

Biology of Demyelinating Diseases

  • Chapter
  • First Online:
Neurochemical Mechanisms in Disease

Part of the book series: Advances in Neurobiology ((NEUROBIOL,volume 1))

  • 995 Accesses

Abstract

Demyelinating diseases are those in which myelin is the primary target of damage on the basis of neuroradiological, neuropatholgical, neurochemical, and genetic studies. This review describes the morphological aspects of the myelin sheath which is the most abundant membrane structure in the vertebrate nervous system. It is made of oligodendrocytes in the CNS and Schwann cells in the PNS. It comprises four distinct regions: the node of Ranvier which contains voltage-gated Na+ channels, paranodal loops which are major sites of myelin-axon adhesion, the juxtaparanode, and the internode which is the part of the axon which is ensheathed by a segment of myelin. Demyelination is segmental in the peripheral nervous system and focal in the central nervous system. Myelin is necessary for nerve conduction velocity. Dys- and demyelination can involve specific constituents of the CNS and the PNS both for genetic (leukodystrophies) or acquired diseases. Numerous components are different and differently involved in CNS and PNS myelin, both among proteins and lipids (sphingolipids). Outside of the abnormalities of specific myelin components leading to genetic diseases, experimental models of demyelination (experimental autoimmune encephalomyelitis, cuprizone intoxication, lysolecithin-induced demyelination, and ethidium bromide treatment are also described). During myelin repair, a thinner myelin sheath is produced with shorter internodes and efficient nerve conduction is produced. Dysfunction of astrocytes may be involved in some genetic diseases of myelin. There are many growth factors and transcription factors involved in the process of myelination and demyelination among which eukaryotic initiation factor 2B (elf2B) leading to vanishing white matter disease (CACH). The role of hormones and sexual dimorphism of oligodendrocytes and myelin anre also described. New areas of research are being developed showing the involvement of myelin deficiency in psychiatric diseases and cognition.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 259.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 329.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 329.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Aberg K, Saetre P, Jareborg N, Jazin E (2006) Human QKI, a potential regulator of mRNA expression of human oligodendrocyte-related genes involved in schizophrenia. Proc Natl Acad Sci U S A 103:7482–7487

    Article  PubMed  CAS  Google Scholar 

  • Amaducci L, Sorbi S, Piacentini S, Bick KL (1991) The first Alzheimer disease case: a metachromatic leukodystrophy? Dev Neurosci 13:186–187

    Article  PubMed  CAS  Google Scholar 

  • Arevalo-Martin A, Vela JM, Molina-Holgado E, Borrell J, Guaza C (2003) Therapeutic action of cannabinoids in a murine model of multiple sclerosis. J Neurosci 23:2511–2516

    PubMed  CAS  Google Scholar 

  • Aslin RN, Schlaggar BL (2006) Is myelination the precipitating neural event for language development in infants and toddlers? Neurology 66:304–305

    Article  PubMed  Google Scholar 

  • Aubourg P, Dubois-Dalcq M (2000) X-linked adrenoleukodystrophy enigma: how does the ALD peroxisomal transporter mutation affect CNS glia? Glia 29:186–190

    Article  PubMed  CAS  Google Scholar 

  • Balabanov R, Strand K, Goswami R, McMahon E, Begolka W, Miller SD, Popko B (2007) Interferon-gamma-oligodendrocyte interactions in the regulation of experimental autoimmune encephalomyelitis. J Neurosci 27:2013–2024

    Article  PubMed  CAS  Google Scholar 

  • Bandtlow CE, Schwab ME (2000) NI-35/250/nogo-a: a neurite growth inhibitor restricting structural plasticity and regeneration of nerve fibers in the adult vertebrate CNS. Glia 29:175–181

    Article  PubMed  CAS  Google Scholar 

  • Bansal R, Stefansson K, Pfeiffer SE (1992) Proligodendroblast antigen (POA), a developmental antigen expressed by A007/O4-positive oligodendrocyte progenitors prior to the appearance of sulfatide and galactocerebroside. J Neurochem 58:2221–2229

    Article  PubMed  CAS  Google Scholar 

  • Baumann N (2000) Specificity of antiglycolipid antibodies. Clin Rev Allergy Immunol 19:31–40

    Article  PubMed  CAS  Google Scholar 

  • Baumann N, Pham-Dinh D (2001) Biology of oligodendrocyte and myelin in the mammalian central nervous system. Physiol Rev 81:871–927

    PubMed  CAS  Google Scholar 

  • Baumann N, Pham-Dinh D (2002) The Astrocyte. In: Ramachandran VS (ed) Encyclopaedia of the Human Brain. Academic Press/Elsevier Science, New York, NY, pp 251–268

    Chapter  Google Scholar 

  • Baumann N, Turpin JC (2000) Adult-onset leukodystrophies. J Neurol 247:751–759

    Article  PubMed  CAS  Google Scholar 

  • Bebo BF Jr, Zelinka-Vincent E, Adamus G, Amundson D, Vandenbark AA, Offner H (1998) Gonadal hormones influence the immune response to PLP 139–151 and the clinical course of relapsing experimental autoimmune encephalomyelitis. J Neuroimmunol 84:122–130

    Article  PubMed  CAS  Google Scholar 

  • Beckman M (2004) Crime, culpability and the adolescent brain. Science 305:596–599

    Article  PubMed  CAS  Google Scholar 

  • Biffiger K, Bartsch S, Montag D, Aguzzi A, Schachner M, Bartsch U (2000) Severe hypomyelination of the murine CNS in the absence of myelin-associated glycoprotein and fyn tyrosine kinase. J Neurosci 20:7430–7437

    PubMed  CAS  Google Scholar 

  • Billon N, Jolicoeur C, Tokumoto Y, Vennstrom B, Raff M (2002) Normal timing of oligodendrocyte development depends on thyroid hormone receptor alpha 1 (TRalpha1). EMBO J 21:6452–6460

    Article  PubMed  CAS  Google Scholar 

  • Bjartmar C, Trapp BD (2001) Axonal and neuronal degeneration in multiple sclerosis: mechanisms and functional consequences. Curr Opin Neurol 14:271–278

    Article  PubMed  CAS  Google Scholar 

  • Bjartmar C, Yin X, Trapp BD (1999) Axonal pathology in myelin disorders. J Neurocytol 28:383–395

    Article  PubMed  CAS  Google Scholar 

  • Blakemore WF (1973a) Remyelination of the superior cerebellar peduncle in the mouse following demyelination induced by feeding cuprizone. J Neurol Sci 20:73–83

    Article  PubMed  CAS  Google Scholar 

  • Blakemore WF (1973b) Demyelination of the superior cerebellar peduncle in the mouse induced by cuprizone. J Neurol Sci 20:63–72

    Article  PubMed  CAS  Google Scholar 

  • Bolton C, Paul C (2006) Glutamate receptors in neuroinflammatory demyelinating disease. Mediators Inflamm 2006:93684

    Article  PubMed  CAS  Google Scholar 

  • Bradl M, Hohfeld R (2003) Molecular pathogenesis of neuroinflammation. J Neurol Neurosurg Psychiatry 74:1364–1370

    Article  PubMed  CAS  Google Scholar 

  • Bradl M, Linington C (1996) Animal models of demyelination. Brain Pathol 6:303–311

    Article  PubMed  CAS  Google Scholar 

  • Brenner M, Johnson AB, Boespflug-Tanguy O, Rodriguez D, Goldman JE, Messing A (2001) Mutations in GFAP, encoding glial fibrillary acidic protein, are associated with Alexander disease. Nat Genet 27:117–120

    Article  PubMed  CAS  Google Scholar 

  • Bugga L, Gadient RA, Kwan K, Stewart CL, Patterson PH (1998) Analysis of neuronal and glial phenotypes in brains of mice deficient in leukemia inhibitory factor. J Neurobiol 36:509–524

    Article  PubMed  CAS  Google Scholar 

  • Buschard K, Fredman P, Bog-Hansen E, Blomqvist M, Hedner J, Rastam L, Lindblad U (2005) Low serum concentration of sulfatide and presence of sulfated lactosylceramid are associated with Type 2 diabetes. The Skaraborg Project. Diabet Med 22:1190–1198

    Article  PubMed  CAS  Google Scholar 

  • Butt AM, Kiff J, Hubbard P, Berry M (2002) Synantocytes: new functions for novel NG2 expressing glia. J Neurocytol 31:551–565

    Article  PubMed  CAS  Google Scholar 

  • Calza L, Fernandez M, Giuliani A, Aloe L, Giardino L (2002) Thyroid hormone activates oligodendrocyte precursors and increases a myelin-forming protein and NGF content in the spinal cord during experimental allergic encephalomyelitis. Proc Natl Acad Sci U S A 99:3258–3263

    Article  PubMed  CAS  Google Scholar 

  • Camara J, Ffrench-Constant C (2007) Lessons from oligodendrocyte biology on promoting repair in multiple sclerosis. J Neurol 254(Suppl 1):I15–I22

    Article  CAS  Google Scholar 

  • Campagnoni AT, Campagnoni CW, Bourre JM, Jacque C, Baumann N (1984) Cell-free synthesis of myelin basic proteins in normal and dysmyelinating mutant mice. J Neurochem 42:733–739

    Article  PubMed  CAS  Google Scholar 

  • Campagnoni AT, Macklin WB (1988) Cellular and molecular aspects of myelin protein gene expression. Mol Neurobiol 2:41–89

    Article  PubMed  CAS  Google Scholar 

  • Campagnoni AT, Skoff RP (2001) The pathobiology of myelin mutants reveals novel biological functions of the MBP and PLP-1 genes. Brain Pathol 11:74–91

    Article  PubMed  CAS  Google Scholar 

  • Cao Z, Qiu J, Domeniconi M, Hou J, Bryson JB, Mellado W, Filbin MT (2007) The inhibition site on myelin-associated glycoprotein is within Ig-domain 5 and is distinct from the sialic acid binding site. J Neurosci 27:9146–9154

    Article  PubMed  CAS  Google Scholar 

  • Capello E, Voskuhl RR, McFarland HF, Raine CS (1997) Multiple sclerosis: re-expression of a developmental gene in chronic lesions correlates with remyelination. Ann Neurol 41:797–805

    Article  PubMed  CAS  Google Scholar 

  • Cerghet M, Skoff RP, Bessert D, Zhang Z, Mullins C, Ghandour MS (2006) Proliferation and death of oligodendrocytes and myelin proteins are differentially regulated in male and female rodents. J Neurosci 26:1439–1447

    Article  PubMed  CAS  Google Scholar 

  • Charles P, Hernandez MP, Stankoff B, Aigrot MS, Colin C, Rougon G, Zalc B, Lubetzki C (2000) Negative regulation of central nervous system myelination by polysialylated-neural cell adhesion molecule. Proc Natl Acad Sci U S A 97:7585–7590

    Article  PubMed  CAS  Google Scholar 

  • Charles P, Reynolds R, Seilhean D, Rougon G, Aigrot MS, Niezgoda A, Zalc B, Lubetzki C (2002) Re-expression of PSA-NCAM by demyelinated axons: an inhibitor of remyelination in multiple sclerosis? Brain 125:1972–1979

    Article  PubMed  Google Scholar 

  • Chassande B, Leger JM, Younes-Chennoufi AB, Bengoufa D, Maisonobe T, Bouche P, Baumann N (1998) Peripheral neuropathy associated with IgM monoclonal gammopathy: correlations between M-protein antibody activity and clinical/electrophysiological features in 40 cases. Muscle Nerve 21:55–62

    Article  PubMed  CAS  Google Scholar 

  • Chow E, Mottahedeh J, Prins M, Ridder W, Nusinowitz S, Bronstein JM (2005) Disrupted compaction of CNS myelin in an OSP/Claudin-11 and PLP-1/DM20 double knockout mouse. Mol Cell Neurosci 29:405–413

    Article  PubMed  CAS  Google Scholar 

  • Chun SJ, Rasband MN, Sidman RL, Habib AA, Vartanian T (2003) Integrin-linked kinase is required for laminin-2-induced oligodendrocyte cell spreading and CNS myelination. J Cell Biol 163:397–408

    Article  PubMed  CAS  Google Scholar 

  • Coetzee T, Suzuki K, Popko B (1998) New perspectives on the function of myelin galactolipids. Trends Neurosci 21:126–130

    Article  PubMed  CAS  Google Scholar 

  • Collarini EJ, Pringle N, Mudhar H, Stevens G, Kuhn R, Monuki ES, Lemke G, Richardson WD (1991) Growth factors and transcription factors in oligodendrocyte development. J Cell Sci Suppl 15:117–123

    PubMed  CAS  Google Scholar 

  • Colognato H, Ffrench-Constant C, Feltri ML (2005) Human diseases reveal novel roles for neural laminins. Trends Neurosci 28:480–486

    Article  PubMed  CAS  Google Scholar 

  • Colognato H, Galvin J, Wang Z, Relucio J, Nguyen T, Harrison D, Yurchenco PD, Ffrench-Constant. C (2007) Identification of dystroglycan as a second laminin receptor in oligodendrocytes, with a role in myelination. Development 134:1723–1736

    Article  PubMed  CAS  Google Scholar 

  • Colsch B, Afonso C, Popa I, Portoukalian J, Fournier F, Tabet JC, Baumann N (2004) Characterization of the ceramide moieties of sphingoglycolipids from mouse brain by ESI-MS/MS: identification of ceramides containing sphingadienine. J Lipid Res 45:281–286

    Article  PubMed  CAS  Google Scholar 

  • Colsch B, Baumann N, Ghandour S (2008) Generation and characterization of the binding epitope of a novel monoclonal antibody to sulfatide (sulfogalactosylceramide) OL-2: applications of antigen immunodetections in brain tissues and urinary samples. J Neuroimmunol 193:52–58

    Article  PubMed  CAS  Google Scholar 

  • Coman I, Aigrot MS, Seilhean D, Reynolds R, Girault JA, Zalc B, Lubetzki C (2006) Nodal, paranodal and juxtaparanodal axonal proteins during demyelination and remyelination in multiple sclerosis. Brain 129:3186–3195

    Article  PubMed  CAS  Google Scholar 

  • Coman I, Barbin G, Charles P, Zalc B, Lubetzki C (2005) Axonal signals in central nervous system myelination, demyelination and remyelination. J Neurol Sci 233:67–71

    Article  PubMed  CAS  Google Scholar 

  • Craner MJ, Hains BC, Lo AC, Black JA, Waxman SG (2004a) Co-localization of sodium channel Nav1.6 and the sodium-calcium exchanger at sites of axonal injury in the spinal cord in EAE. Brain 127:294–303

    Article  PubMed  Google Scholar 

  • Craner MJ, Newcombe J, Black JA, Hartle C, Cuzner ML, Waxman SG (2004b) Molecular changes in neurons in multiple sclerosis: altered axonal expression of Nav1.2 and Nav1.6 sodium channels and Na+/Ca2+ exchanger. Proc Natl Acad Sci U S A 101:8168–8173

    Article  PubMed  CAS  Google Scholar 

  • Craves FB, Zalc B, Leybin L, Baumann N, Loh HH (1980) Antibodies to cerebroside sulfate inhibit the effects of morphine and beta-endorphin. Science 207:75–76

    Article  PubMed  CAS  Google Scholar 

  • Cui Y, Colsch B, Afonso C, Baumann N, Tabet JC, Mallet JM, Zhang Y (2008) Synthetic sulfogalactosylceramide (sulfatide) and its use for the mass spectrometric quantitative urinary determination in metachrompatic leukodystrophies. Glycoconj J 25:147–155

    Article  PubMed  CAS  Google Scholar 

  • Delarasse C, Daubas P, Mars LT, Vizler C, Litzenburger T, Iglesias A, Bauer J, Della Gaspera B, Schubart A, Decker L, Dimitri D, Roussel G, Dierich A, Amor S, Dautigny A, Liblau R, Pham-Dinh D (2003) Myelin/oligodendrocyte glycoprotein-deficient (MOG-deficient) mice reveal lack of immune tolerance to MOG in wild-type mice. J Clin Invest 112:544–553

    PubMed  CAS  Google Scholar 

  • Delarasse C, Della Gaspera B, Lu CW, Lachapelle F, Gelot A, Rodriguez D, Dautigny A, Genain C, Pham-Dinh D (2006) Complex alternative splicing of the myelin oligodendrocyte glycoprotein gene is unique to human and non-human primates. J Neurochem 98:1707–1707

    Article  PubMed  CAS  Google Scholar 

  • Demerens C, Stankoff B, Logak M, Anglade P, Allinquant B, Couraud F, Zalc B, Lubetzki C (1996) Induction of myelination in the central nervous system by electrical activity. Proc Natl Acad Sci U S A 93:9887–9892

    Article  PubMed  CAS  Google Scholar 

  • Der Perng M, Su M, Wen SF, Li R, Gibbon T, Prescott AR, Brenner M, Quinlan RA (2006) The Alexander disease-causing glial fibrillary acidic protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of alpha B-crystallin and HSP27. Am J Hum Genet 79:197–213

    Article  CAS  Google Scholar 

  • Dietrich J, Lacagnina M, Gass D, Richfield E, Mayer-Proschel M, Noble M, Torres C, Proschel C (2005) EIF2B5 mutations compromise GFAP+ astrocyte generation in vanishing white matter leukodystrophy. Nat Med 11:277–283

    Article  PubMed  CAS  Google Scholar 

  • Dubourg O (2004) Charcot-Marie-Tooth disease: from phenotype to genotype. Rev Neurol (Paris) 160:1221–1229

    CAS  Google Scholar 

  • Duncan ID (2005) The PLP-1 mutants from mouse to man. J Neurol Sci 228:204–205

    Article  PubMed  CAS  Google Scholar 

  • Duncan ID, Nadon NL, Hoffman RL, Lunn KF, Csiza C, Wells MR (1995) Oligodendrocyte survival and function in the long-lived strain of the myelin deficient rat. J Neurocytol 24:745–762

    Article  PubMed  CAS  Google Scholar 

  • Dupouey P, Jacque C, Bourre JM, Cesselin F, Privat A, Baumann N (1979) Immunochemical studies of myelin basic protein in shiverer mouse devoid of major dense line of myelin. Neurosci Lett 12:113–118

    Article  PubMed  CAS  Google Scholar 

  • Dupree JL, Girault JA, Popko B (1999) Axo-glial interactions regulate the localization of axonal paranodal proteins. J Cell Biol 147:1145–1152

    Article  PubMed  CAS  Google Scholar 

  • Durand B, Raff M (2000) A cell-intrinsic timer that operates during oligodendrocyte development. Bioessays 22:64–71

    Article  PubMed  CAS  Google Scholar 

  • Edgar JM, McLaughlin M, Yool D, Zhang SC, Fowler JH, Montague P, Barrie JA, McCulloch MC, Duncan ID, Garbern J, Nave KA, Griffiths IR (2004) Oligodendroglial modulation of fast axonal transport in a mouse model of hereditary spastic paraplegia. J Cell Biol 166:121–131

    Article  PubMed  CAS  Google Scholar 

  • Eftekharpour E, Karimi-Abdolrezaee S, Sinha K, Velumian AA, Kwiecien JM, Fehlings MG (2005) Structural and functional alterations of spinal cord axons in adult Long Evans Shaker (LES) dysmyelinated rats. Exp Neurol 193:334–349

    Article  PubMed  CAS  Google Scholar 

  • Eshed Y, Feinberg K, Poliak S, Sabanay H, Sarig-Nadir O, Spiegel I, Bermingham JR Jr, Peles E (2005) Gliomedin mediates Schwann cell-axon interaction and the molecular assembly of the nodes of Ranvier. Neuron 47:215–229

    Article  PubMed  CAS  Google Scholar 

  • Espinosa-Jeffrey A, Zhao P, Awosika W, Wu N, Macias F, Cepeda C, Levine M, De Vellis J (2006) Activation, proliferation and commitment of enogenous stem/progenitor cells to the oligodendrocyte lineage by TS1 in a rat model of dysmyelination. Dev Neurosci 28:488–498

    Article  PubMed  CAS  Google Scholar 

  • Feng JM (2007) Minireview: expression and function of golli protein in immune system. Neurochem Res 32:273–278

    Article  PubMed  CAS  Google Scholar 

  • Fields RD (2006) Nerve impulses regulate myelination through purinergic signalling. Novartis Found Symp 276:148–158; discussion 158–61, 233–7, 275–281

    Article  PubMed  CAS  Google Scholar 

  • Filbin MT (2003) Myelin-associated inhibitors of axonal regeneration in the adult mammalian CNS. Nat Rev Neurosci 4:703–713

    Article  PubMed  CAS  Google Scholar 

  • Filley C (1998) The behavioral neurology of cerebral white matter. Neurology 50:1535–1545

    PubMed  CAS  Google Scholar 

  • Folch J, Lees. M (1951) Proteolipides, a new type of tissue lipoproteins; their isolation from brain. J Biol Chem 191:807–817

    PubMed  CAS  Google Scholar 

  • Fouad K, Klusman I, Schwab ME (2004) Regenerating corticospinal fibers in the Marmoset (Callitrix jacchus) after spinal cord lesion and treatment with the anti-Nogo-A antibody IN-1. Eur J Neurosci 20:2479–2482

    Article  PubMed  CAS  Google Scholar 

  • Franklin RJ (2002) Why does remyelination fail in multiple sclerosis? Nat Rev Neurosci 3:705–714

    Article  PubMed  CAS  Google Scholar 

  • Friedman HC, Jelsma TN, Bray GM, Aguayo AJ (1996) A distinct pattern of trophic factor expression in myelin-deficient nerves of Trembler mice: implications for trophic support by Schwann cells. J Neurosci 16:5344–5350

    PubMed  CAS  Google Scholar 

  • Fushimi S, Shirabe T (2004) Expression of insulin-like growth factors in remyelination following ethidium bromide-induced demyelination in the mouse spinal cord. Neuropathology 24:208–218

    Article  PubMed  Google Scholar 

  • Garbern JY (2005) Pelizaeus-Merzbacher disease: pathogenic mechanisms and insights into the roles of proteolipid protein 1 in the nervous system. J Neurol Sci 228:201–203

    Article  PubMed  CAS  Google Scholar 

  • Garbern JY, Cambi F, Tang XM, Sima AA, Vallat JM, Bosch EP, Lewis R, Shy M, Sohi J, Kraft G, Chen KL, Joshi I, Leonard DG, Johnson W, Raskind W, Dlouhy SR, Pratt V, Hodes ME, Bird T, Kamholz J (1997) Proteolipid protein is necessary in peripheral as well as central myelin. Neuron 19:205–218

    Article  PubMed  CAS  Google Scholar 

  • Garbern JY, Yool DA, Moore GJ, Wilds IB, Faulk MW, Klugmann M, Nave KA, Sistermans EA, van der Knaap MS, Bird TD, Shy ME, Kamholz JA, Griffiths IR (2002) Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination and inflammation. Brain 125:551–561

    Article  PubMed  Google Scholar 

  • Gatewood JD, Morgan MD, Eaton M, McNamara IM, Stevens LF, Macbeth AH, Meyer EA, Lomas LM, Kozub FJ, Lambert KG, Kinsley CH (2005) Motherhood mitigates aging-related decrements in learning and memory and positively affects brain aging in the rat. Brain Res Bull 66:91–98

    Article  PubMed  Google Scholar 

  • Giaume C, Kirchhoff F, Matute C, Reichenbach A, Verkhratsky A (2007) Glia: the fulcrum of brain diseases. Cell Death Differ 14:1324–1335

    Article  PubMed  CAS  Google Scholar 

  • Gieselmann V, Franken S, Klein D, Mansson JE, Sandhoff R, Lullmann Rauch R, Hartmann D, Saravanan VP, De Deyn PP, D’Hooge R, Van Der Linden AM, Schaeren-Wiemers N (2003) Metachromatic leukodystrophy: consequences of sulphatide accumulation. Acta Paediatr Suppl 92:74–79; discussion 45

    Article  PubMed  CAS  Google Scholar 

  • Gillespie CS, Sherman DL, Blair GE, Brophy PJ (1994) Periaxin, a novel protein of myelinating Schwann cells with a possible role in axonal ensheathment. Neuron 12:497–508

    Article  PubMed  CAS  Google Scholar 

  • Givogri MI, Costa RM, Schonmann V, Silva AJ, Campagnoni AT, Bongarzone ER (2002) Central nervous system myelination in mice with deficient expression of Notch1 receptor. J Neurosci Res 67:309–320

    Article  PubMed  CAS  Google Scholar 

  • Goldberg JL, Barres BA (2000) Nogo in nerve regeneration. Nature 403:369–370

    Article  PubMed  CAS  Google Scholar 

  • Gow A, Sharma R (2003) The unfolded protein response in protein aggregating diseases. Neuromolecular Med 4:73–94

    Article  PubMed  CAS  Google Scholar 

  • Gow A, Southwood CM, Lazzarini RA (1998) Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease. J Cell Biol 140:925–934

    Article  PubMed  CAS  Google Scholar 

  • Graham DI, Lantos PA (1997) Greenfield’s Neuropathology, 6th edn. Arnold, London

    Google Scholar 

  • Gregg C, Shikar V, Larsen P, Mak G, Chojnacki A, Yong VW, Weiss S (2007) White matter plasticity and enhanced remyelination in the maternal CNS. J Neurosci 27:1812–1823

    Article  PubMed  CAS  Google Scholar 

  • Hagemann TL, Connor JX, Messing A (2006) Alexander disease-associated glial fibrillary acidic protein mutations in mice induce Rosenthal fiber formation and a white matter stress response. J Neurosci 26:11162–11173

    Article  PubMed  CAS  Google Scholar 

  • Hahn AF, Ainsworth PJ, Bolton CF, Bilbao JM, Vallat JM (2001) Pathological findings in the x-linked form of Charcot-Marie-Tooth disease: a morphometric and ultrastructural analysis. Acta Neuropathol (Berl) 101:129–139

    CAS  Google Scholar 

  • Haroutunian VP, Davis KL (2007) Introduction to the special section: Myelin and oligodendrocyte abnormalities in schizophrenia. Int J Neuropsychopharmacol 10:499–502

    Article  PubMed  CAS  Google Scholar 

  • Haroutunian V, Katsel P, Dracheva S, Stewart DG, Davis KL (2007) Variations in oligodendrocyte-related gene expression across multiple cortical regions: implications for the pathophysiology of schizophrenia. Int J Neuropsychopharmacol 10:565–573

    Article  PubMed  CAS  Google Scholar 

  • Hausler M, Anhuf D, Schuler H, Ramaekers VT, Thron A, Zerres K, Moller-Hartmann W (2005) White-matter disease in 18q deletion (18q-) syndrome: magnetic resonance spectroscopy indicates demyelination or increased myelin turnover rather than dysmyelination. Neuroradiology 47:83–86

    Article  PubMed  CAS  Google Scholar 

  • Honke K, Hirahara Y, Dupree J, Suzuki K, Popko B, Fukushima K, Fukushima J, Nagasawa T, Yoshida N, Wada Y, Taniguchi N (2002) Paranodal junction formation and spermatogenesis require sulfoglycolipids. Proc Natl Acad Sci U S A 99:4227–4232

    Article  PubMed  CAS  Google Scholar 

  • Howell OW, Palser A, Polito A, Melrose S, Zonta B, Scheiermann C, Vora AJ, Brophy PJ, Reynolds R (2006) Disruption of neurofascin localization reveals early changes preceding demyelination and remyelination in multiple sclerosis. Brain 129:3173–3185

    Article  PubMed  CAS  Google Scholar 

  • Ilyas AA, Chen ZW, Cook SD (2003) Antibodies to sulfatide in cerebrospinal fluid of patients with multiple sclerosis. J Neuroimmunol 139:76–80

    Article  PubMed  CAS  Google Scholar 

  • Ishibashi T, Dakin KA, Stevens B, Lee PR, Kozlov SV, Stewart CL, Fields RD (2006) Astrocytes promote myelination in response to electrical impulses. Neuron 49:823–832

    Article  PubMed  CAS  Google Scholar 

  • Ishibashi T, Ding L, Ikenaka K, Inoue Y, Miyado K, Mekada E, Baba H (2004) Tetraspanin protein CD9 is a novel paranodal component regulating paranodal junctional formation. J Neurosci 24:96–102

    Article  PubMed  CAS  Google Scholar 

  • Ishibashi T, Dupree JL, Ikenaka K, Hirahara Y, Honke K, Peles E, Popko B, Suzuki K, Nishino H, Baba H (2002) A myelin galactolipid, sulfatide, is essential for maintenance of ion channels on myelinated axon but not essential for initial cluster formation. J Neurosci 22:6507–6514

    PubMed  CAS  Google Scholar 

  • Jagannathan NR, Tandon N, Raghunathan P, Kochupillai N (1998) Reversal of abnormalities of myelination by thyroxine therapy in congenital hypothyroidism: localized in vivo proton magnetic resonance spectroscopy (MRS) study. Brain Res Dev Brain Res 109:179–186

    Article  PubMed  CAS  Google Scholar 

  • Jessen KR, Mirsky R (1991) Schwann cell precursors and their development. Glia 4:185–194

    Article  PubMed  CAS  Google Scholar 

  • John GR, Shankar SL, Shafit-Zagardo B, Massimi A, Lee SC, Raine CS, Brosnan CF (2002) Multiple sclerosis: re-expression of a developmental pathway that restricts oligodendrocyte maturation. Nat Med 8:1115–1121

    Article  PubMed  CAS  Google Scholar 

  • Jung-Testas I, Do Thi A, Koenig H, Desarnaud F, Shazand K, Schumacher M, Baulieu EE (1999) Progesterone as a neurosteroid: synthesis and actions in rat glial cells. J Steroid Biochem Mol Biol 69:97–107

    Article  PubMed  CAS  Google Scholar 

  • Kaga Y, Shoemaker WJ, Furusho M, Bryant M, Rosenbluth J, Pfeiffer SE, Oh L, Rasband M, Lappe-Siefke C, Yu K, Ornitz DM, Nave KA, Bansal R (2006) Mice with conditional inactivation of fibroblast growth factor receptor-2 signaling in oligodendrocytes have normal myelin but display dramatic hyperactivity when combined with Cnp1 inactivation. J Neurosci 26:12339–12350

    Article  PubMed  CAS  Google Scholar 

  • Kanter JL, Narayana S, Ho PP, Catz I, Warren KG, Sobel RA, Steinman L, Robinson WH (2006) Lipid microarrays identify key mediators of autoimmune brain inflammation. Nat Med 12:138–143

    Article  PubMed  CAS  Google Scholar 

  • Karadottir R, Attwell D (2007) Neurotransmitter receptors in the life and death of oligodendrocytes. Neuroscience 145:1426–1438

    Article  PubMed  CAS  Google Scholar 

  • Karim SA, Barrie JA, McCulloch MC, Montague P, Edgar JM, Kirkham D, Anderson TJ, Nave KA, Griffiths IR, McLaughlin M (2007) PLP-1 overexpression perturbs myelin protein composition and myelination in a mouse model of Pelizaeus-Merzbacher disease. Glia 55:341–351

    Article  PubMed  Google Scholar 

  • Karlsson KA, Samuelsson BE, Steen GO (1974) The lipid composition and Na+-K+-dependent adenosine-triphosphatase activity of the salt (nasal) gland of eider duck and herring gull. A role for sulphatides in sodium-ion transport. Eur J Biochem 46:243–258

    Article  PubMed  CAS  Google Scholar 

  • Karoutzou G, Emrich HM, Dietrich DE (2007) The myelin-pathogenesis puzzle in schizophrenia: a literature review. Mol Psychiatry 13(3):245–260

    Article  PubMed  CAS  Google Scholar 

  • Keirstead HS, Levine JM, Blakemore WF (1998) Response of the oligodendrocyte progenitor cell population (defined by NG2 labelling) to demyelination of the adult spinal cord. Glia 22:161–170

    Article  PubMed  CAS  Google Scholar 

  • Kubicki M, McCarley RW, Shenton ME (2005) Evidence for white matter abnormalities in schizophrenia. Curr Opin Psychiatry 18:121–134

    Article  PubMed  Google Scholar 

  • Kuerten S, Kostova-Bales DA, Frenzel LP, Tigno JT, Tary-Lehmann M, Angelov DN, Lehmann PV (2007) MP4- and MOG:35–55-induced EAE in C57BL/6 mice differentially targets brain, spinal cord and cerebellum. J Neuroimmunol 189:31–40

    Article  PubMed  CAS  Google Scholar 

  • Kuerten S, Lichtenegger FS, Faas S, Angelov DN, Tary-Lehmann M, Lehmann PV (2006) MBP-PLP-1 fusion protein-induced EAE in C57BL/6 mice. J Neuroimmunol 177:99–111

    Article  PubMed  CAS  Google Scholar 

  • Lalive PH, Menge T, Delarasse C, Della Gaspera B, Pham-Dinh D, Villoslada P, von Budingen HC, Genain CP (2006) Antibodies to native myelin oligodendrocyte glycoprotein are serologic markers of early inflammation in multiple sclerosis. Proc Natl Acad Sci U S A 103:2280–2285

    Article  PubMed  CAS  Google Scholar 

  • Larocque D, Richard S (2005) QUAKING KH domain proteins as regulators of glial cell fate and myelination. RNA Biol 2:37–40

    Article  PubMed  CAS  Google Scholar 

  • Lassmann H (2004) Experimental autoimmune encephalomyelitis. In: LazzariniRA(ed) Myelin Biology and Disorders. Elsevier Academic Press, San Diego, CA, pp 1039–1071

    Google Scholar 

  • Lazzarini R (2004) Myelin biology and disorders. Elsevier Academic Press, San Diego, CA

    Google Scholar 

  • Leegwater PA, Vermeulen G, Konst AA, Naidu S, Mulders J, Visser A, Kersbergen P, Mobach D, Fonds D, van Berkel CG, Lemmers RJ, Frants RR, Oudejans CB, Schutgens RB, Pronk JC, van der Knaap MS (2001) Subunits of the translation initiation factor eIF2B are mutant in leukoencephalopathy with vanishing white matter. Nat Genet 29:383–388

    Article  PubMed  CAS  Google Scholar 

  • Ligon KL, Fancy SP, Franklin RJ, Rowitch DH (2006) Olig gene function in CNS development and disease. Glia 54:1–10

    Article  PubMed  Google Scholar 

  • Lindholm E, Ekholm B, Shaw S, Jalonen P, Johansson G, Pettersson U, Sherrington R, Adolfsson R, Jazin E (2001) A schizophrenia-susceptibility locus at 6q25, in one of the world’s largest reported pedigrees. Am J Hum Genet 69:96–105

    Article  PubMed  CAS  Google Scholar 

  • Lopate G, Pestronk A, Evans S, Li L, Clifford D (2005) Anti-sulfatide antibodies in HIV-infected individuals with sensory neuropathy. Neurology 64:1632–1634

    Article  PubMed  CAS  Google Scholar 

  • Lubetzki C, Demerens C, Anglade P, Villarroya H, Frankfurter A, Lee VM, Zalc B (1993) Even in culture, oligodendrocytes myelinate solely axons. Proc Natl Acad Sci U S A 90:6820–6824

    Article  PubMed  CAS  Google Scholar 

  • Lubetzki C, Stankoff B (2000) Role of axonal signals in myelination of the central nervous system. Pathol Biol (Paris) 48:63–69

    CAS  Google Scholar 

  • Lubetzki C, Williams A, Stankoff B (2005) Promoting repair in multiple sclerosis: problems and prospects. Curr Opin Neurol 18:237–244

    Article  PubMed  CAS  Google Scholar 

  • Ludwin SK (1978) Central nervous system demyelination and remyelination in the mouse: an ultrastructural study of cuprizone toxicity. Lab Invest 39:597–612

    PubMed  CAS  Google Scholar 

  • Ludwin SK (1994) Central nervous system remyelination: studies in chronically damaged tissue. Ann Neurol 36(Suppl):S143–S145

    Article  PubMed  Google Scholar 

  • Macklin WB, Campagnoni CW, Deininger PL, Gardinier MV (1987) Structure and expression of the mouse myelin proteolipid protein gene. J Neurosci Res 18:383–394

    Article  PubMed  CAS  Google Scholar 

  • Maier O, van der Heide T, Johnson R, de Vries H, Baron W, Hoekstra D (2006) The function of neurofascin155 in oligodendrocytes is regulated by metalloprotease-mediated cleavage and ectodomain shedding. Exp Cell Res 312:500–511

    Article  PubMed  CAS  Google Scholar 

  • Marcus J, Honigbaum S, Shroff S, Honke K, Rosenbluth J, Dupree JL (2006) Sulfatide is essential for the maintenance of CNS myelin and axon structure. Glia 53:372–381

    Article  PubMed  CAS  Google Scholar 

  • Mason JL, Toews A, Hostettler JD, Morell P, Suzuki K, Goldman JE, Matsushima GK (2004) Oligodendrocytes and progenitors become progressively depleted within chronically demyelinated lesions. Am J Pathol 164:1673–1682

    Article  PubMed  Google Scholar 

  • Mastronardi FG, Moscarello MA (2005) Molecules affecting myelin stability: a novel hypothesis regarding the pathogenesis of multiple sclerosis. J Neurosci Res 80:301–308

    Article  PubMed  CAS  Google Scholar 

  • Matsushima GK, Morell P (2001) The neurotoxicant, cuprizone, as a model to study demyelination and remyelination in the central nervous system. Brain Pathol 11:107–116

    Article  PubMed  CAS  Google Scholar 

  • Mattei MG, Alliel PM, Dautigny A, Passage E, Pham-Dinh D, Mattei JF, Jolles P (1986) The gene encoding for the major brain proteolipid (PLP-1) maps on the q-22 band of the human X chromosome. Hum Genet 72:352–353

    Article  PubMed  CAS  Google Scholar 

  • Matute C (2006) Oligodendrocyte NMDA receptors: a novel therapeutic target. Trends Mol Med 12:289–292

    Article  PubMed  CAS  Google Scholar 

  • McInnes LA, Lauriat TL (2006) RNA metabolism and dysmyelination in schizophrenia. Neurosci Biobehav Rev 30:551–561

    Article  PubMed  CAS  Google Scholar 

  • Menichella DM, Goodenough DA, Sirkowski E, Scherer SS, Paul DL (2003) Connexins are critical for normal myelination in the CNS. J Neurosci 23:5963–5973

    PubMed  CAS  Google Scholar 

  • Menn B, Garcia-Verdugo JM, Yaschine C, Gonzalez-Perez O, Rowitch D, Alvarez-Buylla A (2006) Origin of oligodendrocytes in the subventricular zone of the adult brain. J Neurosci 26:7907–7918

    Article  PubMed  CAS  Google Scholar 

  • Meyer zu Horste G, Prukop T, Nave KA, Sereda MW (2006) Myelin disorders: causes and perspectives of Charcot-Marie-Tooth neuropathy. J Mol Neurosci 28:77–88

    Article  PubMed  Google Scholar 

  • Mi S, Hu B, Hahm K, Luo Y, Kam Hui ESZ, Yuan Q, Wong WM, Wang L, Su H, Chu TH, Guo J, Zhang W, So KF, Perpinsky B, Shao Z, Graff C, Garber E, Jung V, Wu EX, Wu W (2007) Lingo-1 antagnonist promotes spinal cord remyelination and axonal integrity in MOG-induced experimental auto-immune encephalomyelitis. Nat Med 13:1228–3123

    Article  PubMed  CAS  Google Scholar 

  • Mi S, Miller RH, Lee X, Scott ML, Shulag-Morskaya S, Shao Z, Chang J, Thill G, Levesque M, Zhang M, Hession C, Sah D, Trapp B, He Z, Jung V, McCoy JM, Pepinsky RB (2005) LINGO-1 negatively regulates myelination by oligodendrocytes. Nat Neurosci 8:745–751

    Article  PubMed  CAS  Google Scholar 

  • Michailov GV, Sereda MW, Brinkmann BG, Fischer TM, Haug B, Birchmeier C, Role L, Lai C, Schwab MH, Nave KA (2004) Axonal neuregulin-1 regulates myelin sheath thickness. Science 304:700–703

    Article  PubMed  CAS  Google Scholar 

  • Mignot C, Boespflug-Tanguy O, Gelot A, Dautigny A, Pham-Dinh D, Rodriguez D (2004) Alexander disease: putative mechanisms of an astrocytic encephalopathy. Cell Mol Life Sci 61:369–385

    Article  PubMed  CAS  Google Scholar 

  • Mignot C, Delarasse C, Escaich S, Della Gaspera B, Noe E, Colucci-Guyon E, Babinet C, Pekny M, Vicart P, Boespflug-Tanguy O, Dautigny A, Rodriguez D, Pham-Dinh D (2007) Dynamics of mutated GFAP aggregates revealed by real-time imaging of an astrocyte model of Alexander disease. Exp Cell Res 313:2766–2779

    Article  PubMed  CAS  Google Scholar 

  • Mignot C, Desguerre I, Burglen L, Hertz-Pannier L, Renaldo F, Gadisseux JF, Gallet S, Pham-Dinh D, Boespflug-Tanguy O, Rodriguez D (2009) Tumor-like enlargement of the optic chiasm in an infant with Alexander disease. Brain Dev 31:244–247

    Article  PubMed  Google Scholar 

  • Moffett JR, Ross B, Arun P, Madhavarao CN, Namboodiri AM (2007) N-Acetylaspartate in the CNS: from neurodiagnostics to neurobiology. Prog Neurobiol 81:89–131

    Article  PubMed  CAS  Google Scholar 

  • Mosser J, Douar AM, Sarde CO, Kioschis P, Feil R, Moser H, Poustka AM, Mandel JL, Aubourg P (1993) Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters. Nature 361:726–730

    Article  PubMed  CAS  Google Scholar 

  • Nait-Oumesmar B, Picard-Riera N, Kerninon C, Decker L, Seilhean D, Hoglinger GU, Hirsch EC, Reynolds R, Baron-Van Evercooren A (2007) Activation of the subventricular zone in multiple sclerosis: evidence for early glial progenitors. Proc Natl Acad Sci U S A 104:4694–4699

    Article  PubMed  CAS  Google Scholar 

  • Nie DY, Ma QH, Law JW, Chia CP, Dhingra NK, Shimoda Y, Yang WL, Gong N, Chen QW, Xu G, Hu QD, Chow PK, Ng YK, Ling EA, Watanabe K, Xu TL, Habib AA, Schachner M, Xiao ZC (2006) Oligodendrocytes regulate formation of nodes of Ranvier via the recognition molecule OMgp. Neuron Glia Biol 2:151–164

    Article  PubMed  Google Scholar 

  • Nishiyama A, Chang A, Trapp BD (1999) NG2+ glial cells: a novel glial cell population in the adult brain. J Neuropathol Exp Neurol 58:1113–1124

    Article  PubMed  CAS  Google Scholar 

  • Oberheim NA, Wang X, Goldman S, Nedergaard M (2006) Astrocytic complexity distinguishes the human brain. Trends Neurosci 29:547–553

    Article  PubMed  CAS  Google Scholar 

  • Occhi S, Zambroni D, Del Carro U, Amadio S, Sirkowski EE, Scherer SS, Campbell KP, Moore SA, Chen ZL, Strickland S, Di Muzio A, Uncini A, Wrabetz L, Feltri ML (2005) Both laminin and Schwann cell dystroglycan are necessary for proper clustering of sodium channels at nodes of Ranvier. J Neurosci 25:9418–9427

    Article  PubMed  CAS  Google Scholar 

  • Ogawa Y, Schafer DP, Horresh I, Bar V, Hales K, Yang Y, Susuki K, Peles E, Stankewich MC, Rasband MN (2006) Spectrins and ankyrinB constitute a specialized paranodal cytoskeleton. J Neurosci 26:5230–5239

    Article  PubMed  CAS  Google Scholar 

  • Orthmann-Murphy JL, Enriquez AD, Abrams CK, Scherer SS (2007) Loss-of-function GJA12/Connexin47 mutations cause Pelizaeus-Merzbacher-like disease. Mol Cell Neurosci 34:629–641

    Article  PubMed  CAS  Google Scholar 

  • Owens GC, Bunge RP (1989) Evidence for an early role for myelin-associated glycoprotein in the process of myelination. Glia 2:119–128

    Article  PubMed  CAS  Google Scholar 

  • Park SK, Miller R, Krane I, Vartanian T (2001a) The erbB2 gene is required for the development of terminally differentiated spinal cord oligodendrocytes. J Cell Biol 154:1245–1258

    Article  PubMed  CAS  Google Scholar 

  • Park SK, Solomon D, Vartanian T (2001b) Growth factor control of CNS myelination. Dev Neurosci 23:327–337

    Article  PubMed  CAS  Google Scholar 

  • Passage E, Norreel JC, Noack-Fraissignes P, Sanguedolce V, Pizant J, Thirion X, Robaglia-Schlupp A, Pellissier JF, Fontes M (2004) Ascorbic acid treatment corrects the phenotype of a mouse model of Charcot-Marie-Tooth disease. Nat Med 10:396–401

    Article  PubMed  CAS  Google Scholar 

  • Peles E, Salzer JL (2000) Molecular domains of myelinated axons. Curr Opin Neurobiol 10:558–565

    Article  PubMed  CAS  Google Scholar 

  • Pham-Dinh D, Dautigny A, Linington C (2004) The myelin/oligodendrocyte glycoprotein (MOG): structure and physiopathological functions. In: LazzariniRA(ed) Myelin Biology and Disorders. Elsevier Academic Press, San Diego, CA, pp 469–498

    Google Scholar 

  • Pham-Dinh D, Mattei MG, Nussbaum JL, Roussel G, Pontarotti P, Roeckel N, Mather IH, Arzt K, Lindahl KF, Dautigny A (1993) Myelin/oligodendrocyte glycoprotein is a member of a subset of the immunoglobulin superfamily encoded within the major histocompatibility complex. Proc Natl Acad Sci U S A 90:7990–7994

    Article  PubMed  CAS  Google Scholar 

  • Picard-Riera N, Decker L, Delarasse C, Goude K, Nait-Oumesmar B, Liblau R, Pham-Dinh D, Barron van Evercooren A (2002) Experimental autoimmune encephalomyelitis mobilizes neural progenitors from the subventricular zone to undergo oligodendrogenesis in adult mice. Proc Natl Acad Sci U S A 99:13211–13216

    Article  PubMed  CAS  Google Scholar 

  • Pittock SJ, Reindl M, Achenbach S, Berger T, Bruck W, Konig F, Morales Y, Lassmann H, Bryant S, Moore SB, Keegan BM, Lucchinetti CF (2007) Myelin oligodendrocyte glycoprotein antibodies in pathologically proven multiple sclerosis: frequency, stability and clinicopathologic correlations. Mult Scler 13:7–16

    Article  PubMed  CAS  Google Scholar 

  • Powers JM (2005) Demyelination in peroxisomal diseases. J Neurol Sci 228:206–207

    Article  PubMed  CAS  Google Scholar 

  • Pujol JC, Soriano-Mas H, Ortiz N, Sebastian-Galles JM, Losilla JM, Deus J (2006) Myelination of language-related areas in the developing brain. Neurology 66:339–343

    Article  PubMed  CAS  Google Scholar 

  • Quarles RH (2007) Myelin-associated glycoprotein (MAG): past, present and beyond. J Neurochem 100:1431–1448

    PubMed  CAS  Google Scholar 

  • Quinlan RA, Brenner M, Goldman JE, Messing A (2007) GFAP and its role in Alexander disease. Exp Cell Res 313:2077–2087

    Article  PubMed  CAS  Google Scholar 

  • Raine CS, Traugott U, Farooq M, Bornstein MB, Norton WT (1981) Augmentation of immune-mediated demyelination by lipid haptens. Lab Invest 45:174–182

    PubMed  CAS  Google Scholar 

  • Rasband MN, Tayler J, Kaga Y, Yang Y, Lappe-Siefke C, Nave KA, Bansal R (2005) CNP is required for maintenance of axon-glia interactions at nodes of Ranvier in the CNS. Glia 50:86–90

    Article  PubMed  Google Scholar 

  • Rauschka H, Colsch B, Baumann N, Wevers R, Schmidbauer M, Krammer M, Turpin JC, Lefevre M, Olivier C, Tardieu S, Krivit W, Moser H, Moser A, Gieselmann V, Zalc B, Cox T, Reuner U, Tylki-Szymanska A, Aboul-Enein F, LeGuern E, Bernheimer H, Berger J (2006) Late-onset metachromatic leukodystrophy: genotype strongly influences phenotype. Neurology 67:859–863

    Article  PubMed  CAS  Google Scholar 

  • Regenold WT, Phatak P, Marano CM, Gearhart L, Viens CH, Hisley KC (2007) Myelin staining of deep white matter in the dorsolateral prefrontal cortex in schizophrenia, bipolar disorder, and unipolar major depression. Psychiatry Res 151:179–188

    Article  PubMed  CAS  Google Scholar 

  • Roach A, Boylan K, Horvath S, Prusiner SB, Hood LE (1983) Characterization of cloned cDNA representing rat myelin basic protein: absence of expression in brain of shiverer mutant mice. Cell 34:799–806

    Article  PubMed  CAS  Google Scholar 

  • Rodriguez D, Gauthier F, Bertini E, Bugiani M, Brenner M, N’Guyen S, Goizet C, Gelot A, Surtees R, Pedespan JM, Hernandorena X, Troncoso M, Uziel G, Messing A, Ponsot G, Pham-Dinh D, Dautigny A, Boespflug-Tanguy O (2001) Infantile Alexander disease: spectrum of GFAP mutations and genotype-phenotype correlation. Am J Hum Genet 69:1134–1140

    Article  PubMed  CAS  Google Scholar 

  • Rodriguez D, Gelot A, Della Gaspera B, Robain O, Ponsot G, Sarlieve LL, Ghandour S, Pompidou A, Dautigny A, Aubourg P, Pham-Dinh D (1999) Increased density of oligodendrocytes in childhood ataxia with diffuse central hypomyelination (CACH) syndrome: neuropathological and biochemical study of two cases. Acta Neuropathol (Berl) 97:469–480

    Article  CAS  Google Scholar 

  • Rogister B, Ben-Hur T, Dubois-Dalcq M (1999) From neural stem cells to myelinating oligodendrocytes. Mol Cell Neurosci 14:287–300

    Article  PubMed  CAS  Google Scholar 

  • Rosenbluth J, Nave KA, Mierzwa A, Schiff R (2006) Subtle myelin defects in PLP-1-null mice. Glia 54:172–182

    Article  PubMed  Google Scholar 

  • Rosenbluth J, Schiff R, Liang WL, Dou W (2003) Antibody-mediated CNS demyelination II. Focal spinal cord lesions induced by implantation of an IgM antisulfatide-secreting hybridoma. J Neurocytol 32:265–276

    Article  PubMed  CAS  Google Scholar 

  • Roy K, Murtie JC, El-Khodor BF, Edgar N, Sardi SP, Hooks BM, Benoit-Marand M, Chen C, Moore H, O’Donnell P, Brunner D, Corfas G (2007) Loss of erbB signaling in oligodendrocytes alters myelin and dopaminergic function, a potential mechanism for neuropsychiatric disorders. Proc Natl Acad Sci U S A 104:8131–8136

    Article  PubMed  CAS  Google Scholar 

  • Saravanan K, Schaeren-Wiemers N, Klein D, Sandhoff R, Schwarz A, Yaghootfam A, Gieselmann V, Franken S (2004) Specific downregulation and mistargeting of the lipid raft-associated protein MAL in a glycolipid storage disorder. Neurobiol Dis 16:396–406

    Article  PubMed  CAS  Google Scholar 

  • Schaeren-Wiemers N, Bonnet A, Erb M, Erne B, Bartsch U, Kern F, Mantei N, Sherman D, Suter U (2004) The raft-associated protein MAL is required for maintenance of proper axon–glia interactions in the central nervous system. J Cell Biol 166:731–742

    Article  PubMed  CAS  Google Scholar 

  • Scherer SS, Deschenes SM, Xu YT, Grinspan JB, Fischbeck KH, Paul DL (1995) Connexin32 is a myelin-related protein in the PNS and CNS. J Neurosci 15:8281–8294

    PubMed  CAS  Google Scholar 

  • Schiffmann R, Elroy-Stein O (2006) Childhood ataxia with CNS hypomyelination/vanishing white matter disease – a common leukodystrophy caused by abnormal control of protein synthesis. Mol Genet Metab 88:7–15

    Article  PubMed  CAS  Google Scholar 

  • Schumacher M, Guennoun R, Stein DG, De Nicola AF (2007) Progesterone: therapeutic opportunities for neuroprotection and myelin repair. Pharmacol Ther 116:77–106

    Article  PubMed  CAS  Google Scholar 

  • Seldon HL (2007) Extended neocortical maturation time encompasses speciation, fatty acid and lateralization theories of the evolution of schizophrenia and creativity. Med Hypotheses 69(5):1085–1089

    Article  PubMed  CAS  Google Scholar 

  • Sereda MW, Meyer zu Horste G, Suter U, Uzma N, Nave KA (2003) Therapeutic administration of progesterone antagonist in a model of Charcot-Marie-Tooth disease (CMT-1A). Nat Med 9:1533–1537

    Article  PubMed  CAS  Google Scholar 

  • Shy ME (2004) Charcot-Marie-Tooth disease: an update. Curr Opin Neurol 17:579–585

    Article  PubMed  CAS  Google Scholar 

  • Shy ME, Jani A, Krajewski K, Grandis M, Lewis RA, Li J, Shy RR, Balsamo J, Lilien J, Garbern JY, Kamholz J (2004) Phenotypic clustering in MPZ mutations. Brain 127:371–384

    Article  PubMed  Google Scholar 

  • Simons M, Trajkovic K (2006) Neuron-glia communication in the control of oligodendrocyte function and myelin biogenesis. J Cell Sci 119:4381–4389

    Article  PubMed  CAS  Google Scholar 

  • Smith EJ, Blakemore WF, McDonald WI (1979) Central remyelination restores secure conduction. Nature 280:395–396

    Article  PubMed  CAS  Google Scholar 

  • Southwood CM, Garbern J, Jiang W, Gow A (2002) The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease. Neuron 36:585–596

    Article  PubMed  CAS  Google Scholar 

  • Sow A, Lamant M, Bonny JM, Larvaron P, Piaud O, Lecureuil C, Fontaine I, Saleh MC, Garcia Otin AL, Renou JP, Baron B, Zakin M, Guillou F (2006) Oligodendrocyte differentiation is increased in transferrin transgenic mice. J Neurosci Res 83:403–414

    Article  PubMed  CAS  Google Scholar 

  • Spencer T, Domeniconi M, Cao Z, Filbin MT (2003) New roles for old proteins in adult CNS axonal regeneration. Curr Opin Neurobiol 13:133–139

    Article  PubMed  CAS  Google Scholar 

  • Spiegel I, Peles E (2006) A new player in CNS myelination. Neuron 49:777–778

    Article  PubMed  CAS  Google Scholar 

  • Stangel M, Hartung HP (2002) Remyelinating strategies for the treatment of multiple sclerosis. Prog Neurobiol 68:361–376

    Article  PubMed  CAS  Google Scholar 

  • Staugaitis SM, Smith PR, Colman DR (1990) Expression of myelin basic protein isoforms in nonglial cells. J Cell Biol 110:1719–1727

    Article  PubMed  CAS  Google Scholar 

  • Steinman L (2001) Blockade of gamma interferon might be beneficial in MS. Mult Scler 7:275–276

    PubMed  CAS  Google Scholar 

  • Steinman L, Zamvil SS (2006) How to successfully apply animal studies in experimental allergic encephalomyelitis to research on multiple sclerosis. Ann Neurol 60:12–21

    Article  PubMed  CAS  Google Scholar 

  • Stevens B (2006) Cross-talk between growth factor and purinergic signalling regulates Schwann cell proliferation. Novartis Found Symp 276:162–175; discussion 175–80, 233–237, 275–81

    Article  PubMed  CAS  Google Scholar 

  • Stevens B, Fields RD (2000) Response of Schwann cells to action potentials in development. Science 287:2267–2271

    Article  PubMed  CAS  Google Scholar 

  • Stevens B, Ishibashi T, Chen JF, Fields RD (2004) Adenosine: an activity-dependent axonal signal regulating MAP kinase and proliferation in developing Schwann cells. Neuron Glia Biol 1:23–34

    Article  PubMed  Google Scholar 

  • Stevens B, Porta S, Haak LL, Gallo V, Fields RD (2002) Adenosine: a neuron-glial transmitter promoting myelination in the CNS in response to action potentials. Neuron 36:855–868

    Article  PubMed  CAS  Google Scholar 

  • Stewart DG, Davis KL (2004) Possible contributions of myelin and oligodendrocyte dysfunction to schizophrenia. Int Rev Neurobiol 59:381–424

    Article  PubMed  CAS  Google Scholar 

  • Stidworthy MF, Genoud S, Li WW, Leone DP, Mantei N, Suter U, Franklin RJ (2004) Notch1 and Jagged1 are expressed after CNS demyelination, but are not a major rate-determining factor during remyelination. Brain 127:1928–1941

    Article  PubMed  Google Scholar 

  • Suzuki K (2003) Globoid cell leukodystrophy (Krabbe’s disease): update. J Child Neurol 18:595–603

    Article  PubMed  Google Scholar 

  • Suzuki K, Kikkawa Y (1969) Status spongiosus of CNS and hepatic changes induced by cuprizone (biscyclohexanone oxalyldihydrazone). Am J Pathol 54:307–325

    PubMed  CAS  Google Scholar 

  • Tanaka KF, Takebayashi H, Yamazaki Y, Ono K, Naruse M, Iwasato T, Itohara S, Kato H, Ikenaka K (2007) Murine model of Alexander disease: analysis of GFAP aggregate formation and its pathological significance. Glia 55:617–631

    Article  PubMed  Google Scholar 

  • Taveggia C, Zanazzi G, Petrylak A, Yano H, Rosenbluth J, Einheber S, Xu X, Esper RM, Loeb JA, Shrager P, Chao MV, Falls DL, Role L, Salzer JL (2005) Neuregulin-1 type III determines the ensheathment fate of axons. Neuron 47:681–694

    Article  PubMed  CAS  Google Scholar 

  • Teijido O, Casaroli-Marano R, Kharkovets T, Aguado F, Zorzano A, Palacin M, Soriano E, Martinez A, Estevez R (2007) Expression patterns of MLC1 protein in the central and peripheral nervous systems. Neurobiol Dis 26:532–545

    Article  PubMed  CAS  Google Scholar 

  • Terada N, Baracskay K, Kinter M, Melrose S, Brophy PJ, Boucheix C, Bjartmar C, Kidd G, Trapp BD (2002) The tetraspanin protein, CD9, is expressed by progenitor cells committed to oligodendrogenesis and is linked to beta1 integrin, CD81, and Tspan-2. Glia 40:350–359

    Article  PubMed  Google Scholar 

  • Tian GF, Azmi H, Takano T, Xu Q, Peng W, Lin J, Oberheim N, Lou N, Wang X, Zielke HR, Kang J, Nedergaard M (2005) An astrocytic basis of epilepsy. Nat Med 11:973–981

    PubMed  CAS  Google Scholar 

  • Tian DS, Dong Q, Pan DJ, He Y, Yu ZY, Xie MJ, Wang W (2007) Attenuation of astrogliosis by suppressing of microglial proliferation with the cell cycle inhibitor olomoucine in rat spinal cord injury model. Brain Res 1154:206–214

    Article  PubMed  CAS  Google Scholar 

  • Tian R, Gregor M, Wiche G, Goldman JE (2006) Plectin regulates the organization of glial fibrillary acidic protein in Alexander disease. Am J Pathol 168:888–897

    Article  PubMed  CAS  Google Scholar 

  • Tiwari-Woodruff S, Beltran-Parrazal L, Charles A, Keck T, Vu T, Bronstein J (2006) K+ channel KV3.1 associates with OSP/claudin-11 and regulates oligodendrocyte development. Am J Physiol Cell Physiol 291:C687–C698

    Article  PubMed  CAS  Google Scholar 

  • Tomassini V, Onesti E, Mainero C, Giugni E, Paolillo A, Salvetti M, Nicoletti F, Pozzilli C (2005) Sex hormones modulate brain damage in multiple sclerosis: MRI evidence. J Neurol Neurosurg Psychiatry 76:272–275

    Article  PubMed  CAS  Google Scholar 

  • Tominaga K, Matsuda J, Kido M, Naito E, Yokota I, Toida K, Ishimura K, Suzuki K, Kuroda Y (2004) Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the “twitcher” mouse model of globoid cell leukodystrophy. J Neurosci Res 77:507–516

    Article  PubMed  CAS  Google Scholar 

  • Traka M, Goutebroze L, Denisenko N, Bessa M, Nifli A, Havaki S, Iwakura Y, Fukamauchi F, Watanabe K, Soliven B, Girault JA, Karagogeos D (2003) Association of TAG-1 with Caspr2 is essential for the molecular organization of juxtaparanodal regions of myelinated fibers. J Cell Biol 162:1161–1172

    Article  PubMed  CAS  Google Scholar 

  • Trapp BD, Pfeiffer SE, Anitei M, Kidd G (2004) Cell biology of myelin assembly. In: Lazzarini RA et al (eds) Myelin biology and disorders. Elsevier Academic Press, New York, NY, pp 29–55

    Google Scholar 

  • Turpin JC, Jouas M, Sereni C, Pluot M, Baumann N (1985) Révélation à l’âge adulte d’un cas d’adrénoleucodystrophie familiale. Rev Neurol 141:289–295

    PubMed  CAS  Google Scholar 

  • Uhlenberg B, Schuelke M, Ruschendorf F, Ruf N, Kaindl AM, Henneke M, Thiele H, Stoltenburg-Didinger G, Aksu F, Topaloglu H, Nurnberg P, Hubner C, Weschke B, Gartner J (2004) Mutations in the gene encoding gap junction protein alpha 12 (connexin 46.6) cause Pelizaeus-Merzbacher-like disease. Am J Hum Genet 75:251–260

    Article  PubMed  CAS  Google Scholar 

  • Vesce S, Rossi D, Brambilla L, Volterra A (2007) Glutamate release from astrocytes in physiological conditions and in neurodegenerative disorders characterized by neuroinflammation. Int Rev Neurobiol 82:57–71

    Article  PubMed  CAS  Google Scholar 

  • Voas MG, Lyons DA, Naylor SG, Arana N, Rasband MN, Talbot WS (2007) alphaII-spectrin is essential for assembly of the nodes of Ranvier in myelinated axons. Curr Biol 17:562–568

    Article  PubMed  CAS  Google Scholar 

  • von Budingen HC, Hauser SL, Ouallet JC, Tanuma N, Menge T, Genain CP (2004) Frontline: Epitope recognition on the myelin/oligodendrocyte glycoprotein differentially influences disease phenotype and antibody effector functions in autoimmune demyelination. Eur J Immunol 34:2072–2083

    Article  Google Scholar 

  • von Budingen HC, Menge T, Hauser SL, Genain CP (2006) Restrictive and diversifying elements of the anti-myelin/oligodendrocyte glycoprotein antibody response in primate experimental allergic encephalomyelitis. Immunogenetics 58:122–128

    Article  CAS  Google Scholar 

  • Waxman SG, Bangalore L (2003) Electrophysiological consequences of myelination. In: Lazzarini, RA et al (eds) Myelin Biology and Disorders. Academic Press, New York, NY, pp 117–142

    Google Scholar 

  • Weimbs T, Stoffel W (1992) Proteolipid protein (PLP-1) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues and implications for the membrane topology of PLP-1. Biochemistry 31:12289–12296

    Article  PubMed  CAS  Google Scholar 

  • Williams A, Piaton G, Aigrot MS, Belhadi A, Théaudin M, Petermann F, Thomas JL, Zalc B, Lubetzki C (2007b) Semaphorin A and 3F, key players in myelin repair in multiple sclerosis? Brain 130:2554–2565

    Article  PubMed  Google Scholar 

  • Williams A, Piaton G,, Lubetzki C (2007a) Astrocytes-friends or foes in multiple sclerosis? Glia 55:1300–1312

    Article  PubMed  Google Scholar 

  • Willison HJ, Yuki N (2002) Peripheral neuropathies and anti-glycolipid antibodies. Brain 125:2591–2625

    Article  PubMed  Google Scholar 

  • Wilson HC, Scolding NJ, Raine CS (2006) Co-expression of PDGF alpha receptor and NG2 by oligodendrocyte precursors in human CNS and multiple sclerosis lesions. J Neuroimmunol 176:162–173

    Article  PubMed  CAS  Google Scholar 

  • Wolswijk G (1998) Chronic stage multiple sclerosis lesions contain a relatively quiescent population of oligodendrocyte precursor cells. J Neurosci 18:601–609

    PubMed  CAS  Google Scholar 

  • Woodruff RH, Franklin RJ (1999) Demyelination and remyelination of the caudal cerebellar peduncle of adult rats following stereotaxic injections of lysolecithin, ethidium bromide, and complement/anti-galactocerebroside: a comparative study. Glia 5:216–228

    Article  Google Scholar 

  • Xin M, Yue T, Ma Z, Wu FF, Gow A, Lu QR (2005) Myelinogenesis and axonal recognition by oligodendrocytes in brain are uncoupled in Olig1-null mice. J Neurosci 25:1354–1365

    Article  PubMed  CAS  Google Scholar 

  • Yakovlev PI, Lecours LA (1966) The myelinogenic cycles of regional maturation of the brain. BlacKwell, Oxford

    Google Scholar 

  • Yuki N, Susuki K, Koga M, Nishimoto Y, Odaka M, Hirata K, Taguchi K, Miyatake T, Furukawa K, Kobata T, Yamada M (2004) Carbohydrate mimicry between human ganglioside GM1 and Campylobacter jejuni lipooligosaccharide causes Guillain-Barre syndrome. Proc Natl Acad Sci U S A 101:11404–11409

    Article  PubMed  CAS  Google Scholar 

  • Zalc B, Helwig JJ, Ghandour MS, Sarlieve L (1978) Sulfatide in the kidney: how is this lipid involved in sodium chloride transport? FEBS Lett 92:92–96

    Article  PubMed  CAS  Google Scholar 

  • Zeger M, Popken G, Zhang J, Xuan S, Lu QR, Schwab MH, Nave KA, Rowitch D, D’Ercole AJ, Ye P (2007) Insulin-like growth factor type 1 receptor signaling in the cells of oligodendrocyte lineage is required for normal in vivo oligodendrocyte development and myelination. Glia 55:400–411

    Article  PubMed  Google Scholar 

  • Zhao C, Fancy SP, Magy L, Urwin JE, Franklin RJ (2005) Stem cells, progenitors and myelin repair. J Anat 207:251–258

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

Drs Saïd Ghandour and Jean-Claude Turpin are gratefully acknowledged for their help and advice during the redaction of this manuscript, and Eric Noe for the careful reading of text and references. The research work of the authors is supported by grants from ELA Foundation (European Leukodystrophy Association), Association Jerome Lejeune, and INSERM to DP-D, and ARNC (association pour la recherche en neurochimie) to NB.

Author information

Authors and Affiliations

  1. U676 INSERM, University Denis Diderot, Hopital Robert Debre, 48 Bd Serurier, 75019, Paris, France

    Danielle Pham-Dinh

  2. Laboratoire de Neurochimie, Hôpital de la Salpêtrière, F-75013, Paris, France

    Nicole Baumann

  3. Université Paris 6, F-75000, Paris, France

    Nicole Baumann

Authors
  1. Danielle Pham-Dinh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nicole Baumann
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Nicole Baumann .

Editor information

Editors and Affiliations

  1. Burke Rehabilitation Center, Mamaroneck Ave. 785, White Plains, 10605, New York, USA

    John P. Blass

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Pham-Dinh, D., Baumann, N. (2011). Biology of Demyelinating Diseases. In: Blass, J. (eds) Neurochemical Mechanisms in Disease. Advances in Neurobiology, vol 1. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-7104-3_16

Download citation

Publish with us

Policies and ethics

Search

Navigation