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Journal article
Transactive Response DNA-Binding Protein 43 Burden in Familial Alzheimer Disease and Down Syndrome
Archives of neurology (Chicago), v 66(12), pp 1483-1488
01 Dec 2009
PMID: 20008652
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Objective: To assess the transactive response DNA-binding protein 43 (TDP-43) burden in familial forms of Alzheimer disease (FAD) and Down syndrome (DS) to determine whether TDP-43 inclusions are also present.
Design: Using standard immunohistochemical techniques, we examined brain tissue samples from 42 subjects with FAD and 14 with DS.
Results: We found pathological TDP-43 aggregates in 14.0% of participants (6 of 42 and 2 of 14 participants With FAD and DS, respectively). in both FAD and DS, TDP-43 immunoreactivity did not colocalize with neurofibrillary tangles. Occasionally participants with FAD or DS had TDP-43-positive neuropil threads or dots. Overall, the amygdala was most commonly affected, followed by the hippocampus, with no TDP-43 pathology in neocortical regions. A similar distribution of TDP-43 inclusions is seen in sporadic Alzheimer disease, but it differs from that seen in amyotrophic lateral sclerosis and frontotemporal dementia.
Conclusions: Transactive response DNA-binding protein 43 pathology occurs in FAD and DS, similar to that observed in sporadic Alzheimer disease. Thus, pathological TDP-43 may contribute the cognitive impairments in familial and sporadic forms of Alzheimer disease.
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Details
- Title
- Transactive Response DNA-Binding Protein 43 Burden in Familial Alzheimer Disease and Down Syndrome
- Creators
- Carol F. Lippa - Drexel UniversityAndrea L. Rosso - Drexel UniversityLauren D. Stutzbach - University of Pennsylvania Philadelphia United StatesManuela Neumann - Drexel UniversityVirginia M. -Y. Lee - University of Pennsylvania Philadelphia United StatesJohn Q. Trojanowski - University of Pennsylvania Philadelphia United States
- Publication Details
- Archives of neurology (Chicago), v 66(12), pp 1483-1488
- Publisher
- Amer Medical Assoc
- Number of pages
- 6
- Grant note
- P30AG010124 / NATIONAL INSTITUTE ON AGING; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute on Aging (NIA) AG10124; AG17586 / National Institute on Aging; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute on Aging (NIA) National Institutes of Health; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA
- Resource Type
- Journal article
- Language
- English
- Web of Science ID
- WOS:000272554200007
- Scopus ID
- 2-s2.0-73549109864
- Other Identifier
- 991019312379004721
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- Collaboration types
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Clinical Neurology