TDP-43 pathology in familial British dementia View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2009-08

AUTHORS

Claudia Schwab, Tetsuaki Arai, Masato Hasegawa, Haruhiko Akiyama, Sheng Yu, Patrick L. McGeer

ABSTRACT

Trans-activation-responsive DNA-binding protein 43 (TDP-43) is a component of pathological inclusions in amyotrophic lateral sclerosis and several forms of sporadic and familial frontotemporal lobar degeneration. This has suggested defining a new class of diseases known as TDP-43 proteinopathies. However, it has been reported more recently that TDP-43 positive inclusions occur in other neurodegenerative disorders such as Alzheimer's disease, Dementia with Lewy Bodies and Parkinsonism dementia complex of Guam. Here we report the occurrence of TDP-43 inclusions in one other neurodegenerative disorder: familial British dementia. Using a variety of antibodies against phosphorylated and non-phosphorylated TDP-43 epitopes, we found intense accumulation occurred in the form of dystrophic neurites, neuronal cytoplasmic inclusions and was also occasionally associated with neurofibrillary tangles. Double immunostaining revealed that TDP-43 and tau aggregates were rarely directly colocalized, but co-existed in the same neurons as separate inclusions. Double staining with ubiquitin showed a direct colocalization with TDP-43. The phosphorylation-dependent TDP-43 antibodies proved superior to phosphorylation-independent antibodies in revealing pathological inclusions since the former did not stain non-phosphorylated TDP-43 in normal nuclei. Our results support the concept that TDP-43 pathology is not narrowly restricted, but is involved in the etiology of many neurodegenerative disorders. More... »

PAGES

303-311

References to SciGraph publications

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  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1007/s00401-009-0514-3

    DOI

    http://dx.doi.org/10.1007/s00401-009-0514-3

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1019176236

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/19283396


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