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- Title
Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases.
- Authors
Geser, Felix; Martinez-Lage, Maria; Kwong, Linda K.; Lee, Virginia M.-Y.; Trojanowski, John Q.
- Abstract
Ever since the significance of pathological 43-kDa transactivating responsive sequence DNA-binding protein (TDP-43) for human disease has been recognized in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin positive inclusions (FTLD-U), a number of publications have emerged reporting on this pathology in a variety of neurodegenerative diseases. Given the heterogeneous and, in part, conflicting nature of the recent findings, we here review pathological TDP-43 and its relationship to human disease with a special focus on ALS and FTLD-U. To this end, we propose a classification scheme in which pathological TDP-43 is the major disease defining pathology in one group, or is present in addition to other neurodegenerative hallmark pathologies in a second category. We conclude that the TDP-43 proteinopathies represent a novel class of neurodegenerative disorders akin to α-synucleinopathies and tauopathies, with the concept of ALS and FTLD-U to be widened to a broad clinico-pathological multisystem disease, i.e., TDP-43 proteinopathy.
- Subjects
AMYOTROPHIC lateral sclerosis; DEMENTIA; DNA-binding proteins; NEURODEGENERATION; UBIQUITIN; NEUROBIOLOGY
- Publication
Journal of Neurology, 2009, Vol 256, Issue 8, p1205
- ISSN
0340-5354
- Publication type
Article
- DOI
10.1007/s00415-009-5069-7