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Cited by 324

The DOI matches its Crossref record; 92 of the 92 references it cites were found in a registry or an index.

Multiple system atrophy: A primary oligodendrogliopathy

Wenning, Gregor K., Stefanova, Nadia, Jellinger, Kurt A., Poewe, Werner, Schlossmacher, Michael G.·2008·Annals of Neurology

Abstract

To this day, the cause of multiple system atrophy (MSA) remains stubbornly enigmatic. A growing body of observations regarding the clinical, morphological, and biochemical phenotypes of MSA has been published, but the interested student is still left without a clue as to its underlying cause. MSA has long been considered a rare cousin of Parkinson's disease and cerebellar degeneration; it is rich in acronyms but poor in genetic and environmental leads. Because of the worldwide research efforts conducted over the last two decades and the discovery of the α‐synuclein–encoding SNCA gene as a cause of rare familial Parkinson's disease, the MSA field has seen advances on three fronts: the identification of its principal cellular target, that is, oligodendrocytes; the characterization of α‐synuclein–rich glial cytoplasmic inclusions as a suitable marker at autopsy; and improved diagnostic accuracy in living patients resulting from detailed clinicopathological studies. The working model of MSA as a primary glial disorder was recently strengthened by the finding of dysregulation in the metabolism of myelin basic protein and p25α, a central nervous system–specific phosphoprotein (also called tubulin polymerization promoting protein, TPPP). Intriguingly, in early cases of MSA, the oligodendrocytic changes in myelin basic protein and p25α processing were recorded even before formation of glial cytoplasmic inclusions became detectable. Here, we review the evolving concept that MSA may not just be related to Parkinson's disease but also share traits with the family of demyelinating disorders. Although these syndromes vary in their respective cause of oligodendrogliopathy, they have in common myelin disruption that is often followed by axonal dysfunction. Ann Neurol 2008;64:239–246

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Metadata

Authors
Wenning, G. K., Stefanova, N., Jellinger, K. A., Poewe, W., & Schlossmacher, M. G.
Venue
Annals of Neurology
Publisher
Wiley
Published
2008-09-28
Volume
64
Issue
3
Pages
239-246
ISSN
  • 0364-5134 (ISSN-L)
  • 1531-8249
OpenAlex
W2103976813
Cited by
324
References
92
FWCI
13.75
Type
journal article
Language
en
Open access
closed

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CoChat. (2026). Citation check: Multiple system atrophy: A primary oligodendrogliopathy. https://app.cochat.ai/papers/10.1002/ana.21465
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Wenning, G. K., Stefanova, N., Jellinger, K. A., Poewe, W., & Schlossmacher, M. G. (2008). Multiple system atrophy: A primary oligodendrogliopathy. Annals of Neurology. https://doi.org/10.1002/ana.21465

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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