Axonal inclusions in spinocerebellar ataxia type 3 View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2010-07-16

AUTHORS

Kay Seidel, Wilfred F. A. den Dunnen, Christian Schultz, Henry Paulson, Stefanie Frank, Rob A. de Vos, Ewout R. Brunt, Thomas Deller, Harm H. Kampinga, Udo Rüb

ABSTRACT

Protein aggregation is a major pathological hallmark of many neurodegenerative disorders including polyglutamine diseases. Aggregation of the mutated form of the disease protein ataxin-3 into neuronal nuclear inclusions is well described in the polyglutamine disorder spinocerebellar ataxia type 3 (SCA3 or Machado–Joseph disease), although these inclusions are not thought to be directly pathogenic. Neuropil aggregates have not yet been described in SCA3. We performed a systematic immunohistochemical study of serial thick sections through brains of seven clinically diagnosed and genetically confirmed SCA3 patients. Using antibodies against ataxin-3, p62, ubiquitin, the polyglutamine marker 1C2 as well as TDP-43, we analyzed neuronal localization, composition and distribution of aggregates within SCA3 brains. The analysis revealed widespread axonal aggregates in fiber tracts known to undergo neurodegeneration in SCA3. Similar to neuronal nuclear inclusions, the axonal aggregates were ubiquitinated and immunopositive for the proteasome and autophagy associated shuttle protein p62, indicating involvement of neuronal protein quality control mechanisms. Rare TDP-43 positive axonal inclusions were also observed. Based on the correlation between affected fiber tracts and degenerating neuronal nuclei, we hypothesize that these novel axonal inclusions may be detrimental to axonal transport mechanisms and thereby contribute to degeneration of nerve cells in SCA3. More... »

PAGES

449-460

Journal

TITLE

Acta Neuropathologica

ISSUE

4

VOLUME

120

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00401-010-0717-7

DOI

http://dx.doi.org/10.1007/s00401-010-0717-7

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1034883797

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/20635090


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64 inclusion
65 involvement
66 localization
67 major pathological hallmarks
68 mechanism
69 nerve cells
70 neurodegeneration
71 neurodegenerative disorders
72 neuronal localization
73 neuronal nuclear inclusions
74 neuronal nuclei
75 neuropil aggregates
76 nuclear inclusions
77 nucleus
78 p62
79 pathological hallmark
80 patients
81 polyglutamine diseases
82 proteasome
83 protein aggregation
84 protein ataxin-3
85 protein p62
86 protein quality control mechanisms
87 quality control mechanisms
88 sections
89 serial thick sections
90 spinocerebellar ataxia type 3
91 study
92 systematic immunohistochemical study
93 thick sections
94 tract
95 transport mechanism
96 type 3
97 ubiquitin
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