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Assembly of CNS Myelin in the Absence of Proteolipid Protein

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Klugmann, Matthias, Schwab, Markus H., Pühlhofer, Anja, Schneider, Armin, Zimmermann, Frank, Griffiths, Ian R., Nave, Klaus-Armin·1997·Neuron
DOI 10.1016/s0896-6273(01)80046-5
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Abstract

Two proteolipid proteins, PLP and DM20, are the major membrane components of central nervous system (CNS) myelin. Mutations of the X-linked PLP/DM20 gene cause dysmyelination in mouse and man and result in significant mortality. Here we show that mutant mice that lack expression of a targeted PLP gene fail to exhibit the known dysmyelinated phenotype. Unable to encode PLP/DM20 or PLP-related polypeptides, oligodendrocytes are still competent to myelinate CNS axons of all calibers and to assemble compacted myelin sheaths. Ultrastructurally, however, the electron-dense 'intraperiod' lines in myelin remain condensed, correlating with its reduced physical stability. This suggests that after myelin compaction, PLP forms a stabilizing membrane junction, similar to a "zipper." Dysmyelination and oligodendrocyte death emerge as an epiphenomenon of other PLP mutations and have been uncoupled in the PLP null allele from the risk of premature myelin breakdown.

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Metadata

Authors
Klugmann, M., Schwab, M. H., Pühlhofer, A., Schneider, A., Zimmermann, F., Griffiths, I. R., & Nave, K.-A.
Venue
Neuron
Publisher
Elsevier BV
Published
1997-01
Volume
18
Issue
1
Pages
59-70
ISSN
0896-6273 (ISSN-L)
DOI
10.1016/s0896-6273(01)80046-5
OpenAlex
W1976329033
Cited by
471
References
2
FWCI
17.38
Type
journal article
Language
en
Open access
bronze
Licence

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CoChat. (2026). Citation check: Assembly of CNS Myelin in the Absence of Proteolipid Protein. https://app.cochat.ai/papers/10.1016/s0896-6273(01)80046-5
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Klugmann, M., Schwab, M. H., Pühlhofer, A., Schneider, A., Zimmermann, F., Griffiths, I. R., & Nave, K.-A. (1997). Assembly of CNS Myelin in the Absence of Proteolipid Protein. Neuron. https://doi.org/10.1016/s0896-6273(01)80046-5

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