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  Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination and inflammation

Garbern, J. Y., Yool, D. A., Moore, G. J., Wilds, I. B., Faulk, M. W., Klugmann, M., et al. (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(3), 551-561. doi:10.1093/brain/awf043.

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Garbern et al, 2002.pdf (Verlagsversion), 558KB
 
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 Urheber:
Garbern, J. Y., Autor
Yool, D. A., Autor
Moore, G. J., Autor
Wilds, I. B., Autor
Faulk, M. W., Autor
Klugmann, M.1, Autor           
Nave, Klaus-Armin1, Autor           
Sistermans, E. A., Autor
van der Knaap, M. S., Autor
Bird, T. D., Autor
Shy, M. E., Autor
Kamholz, J. A., Autor
Griffiths, I. R., Autor
Affiliations:
1Neurogenetics, Max Planck Institute of Experimental Medicine, Max Planck Society, ou_2173664              

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Schlagwörter: Pelizaeus-Merzbacher disease; axonal; proteolipid protein 1; DM20; N-acetyl aspartate; magnetic resonance spectroscopy
 Zusammenfassung: Axonal degeneration contributes to clinical disability in the acquired demyelinating disease multiple sclerosis. Axonal degeneration occurs during acute attacks, associated with inflammation, and during the chronic progressive phase of the disease in which inflammation is not prominent. To explore the importance of interactions between oligodendrocytes and axons in the CNS, we analysed the brains of rodents and humans with a null mutation in the gene encoding the major CNS myelin protein, proteolipid protein (PLP1, previously PLP). Histological analyses of the CNS of Plp1 null mice and of autopsy material from patients with null PLP1 mutations were performed to evaluate axonal and myelin integrity. In vivo proton magnetic resonance spectroscopy (MRS) of PLP1 null patients was conducted to measure levels of N-acetyl aspartate (NAA), a marker of axonal integrity. Length-dependent axonal degeneration without demyelination was identified in the CNS of Plp1 null mice. Proton MRS of PLP1-deficient patients showed reduced NAA levels, consistent with axonal loss. Analysis of patients' brain tissue also demonstrated a length-dependent pattern of axonal loss without significant demyelination. Therefore, axonal degeneration occurs in humans as well as mice lacking the major myelin protein PLP1. This degeneration is length-dependent, similar to that found in the PNS of patients with the inherited demyelinating neuropathy, CMT1A, but is not associated with significant demyelination. Disruption of PLP1- mediated axonal-glial interactions thus probably causes this axonal degeneration. A similar mechanism may be responsible for axonal degeneration and clinical disability that occur in patients with multiple sclerosis.

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Sprache(n): eng - English
 Datum: 2002-03
 Publikationsstatus: Erschienen
 Seiten: -
 Ort, Verlag, Ausgabe: -
 Inhaltsverzeichnis: -
 Art der Begutachtung: Expertenbegutachtung
 Identifikatoren: DOI: 10.1093/brain/awf043
 Art des Abschluß: -

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Titel: Brain
  Andere : Brain
Genre der Quelle: Zeitschrift
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Ort, Verlag, Ausgabe: London : Macmillan
Seiten: - Band / Heft: 125 (3) Artikelnummer: - Start- / Endseite: 551 - 561 Identifikator: ISSN: 0006-8950
CoNE: https://pure.mpg.de/cone/journals/resource/954925385135