Title |
Topography of FUS pathology distinguishes late-onset BIBD from aFTLD-U
|
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Published in |
Acta Neuropathologica Communications, May 2013
|
DOI | 10.1186/2051-5960-1-9 |
Pubmed ID | |
Authors |
Edward B Lee, Jenny Russ, Hyunjoo Jung, Lauren B Elman, Lama M Chahine, Daniel Kremens, Bruce L Miller, H Branch Coslett, John Q Trojanowski, Vivianna M Van Deerlin, Leo F McCluskey |
Abstract |
Multiple neurodegenerative diseases are characterized by the abnormal accumulation of FUS protein including various subtypes of frontotemporal lobar degeneration with FUS inclusions (FTLD-FUS). These subtypes include atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U), basophilic inclusion body disease (BIBD) and neuronal intermediate filament inclusion disease (NIFID). Despite considerable overlap, certain pathologic features including differences in inclusion morphology, the subcellular localization of inclusions, and the relative paucity of subcortical FUS pathology in aFTLD-U indicate that these three entities represent related but distinct diseases. In this study, we report the clinical and pathologic features of three cases of aFTLD-U and two cases of late-onset BIBD with an emphasis on the anatomic distribution of FUS inclusions. |
X Demographics
Geographical breakdown
Country | Count | As % |
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United Kingdom | 1 | 50% |
United States | 1 | 50% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 2 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Unknown | 20 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Ph. D. Student | 5 | 25% |
Student > Postgraduate | 4 | 20% |
Other | 3 | 15% |
Researcher | 2 | 10% |
Professor > Associate Professor | 2 | 10% |
Other | 1 | 5% |
Unknown | 3 | 15% |
Readers by discipline | Count | As % |
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Neuroscience | 7 | 35% |
Agricultural and Biological Sciences | 4 | 20% |
Biochemistry, Genetics and Molecular Biology | 3 | 15% |
Medicine and Dentistry | 2 | 10% |
Unspecified | 1 | 5% |
Other | 0 | 0% |
Unknown | 3 | 15% |