Molecular neuropathology of TDP-43 proteinopathies.
- Neumann M Institute of Neuropathology, University Hospital of Zurich, Switzerland. Manuela.neumann@usz.ch
- 2009-04-01
Published in:
- International journal of molecular sciences. - 2009
TDP-43
amyotrophic lateral sclerosis
frontotemporal dementia
molecular neuropathology
Amyotrophic Lateral Sclerosis
Animals
DNA-Binding Proteins
Frontotemporal Dementia
Humans
English
The identification of TDP-43 as the major component of the pathologic inclusions in most forms of sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS) resolved a long-standing enigma concerning the nature of the ubiquitinated disease protein under these conditions. Anti-TDP-43 immunohistochemistry and the recent development of novel tools, such as phosphorylation-specific TDP-43 antibodies, have increased our knowledge about the spectrum of pathological changes associated with FTLD-U and ALS and moreover, facilitated the neuropathological routine diagnosis of these conditions. This review summarizes the recent advances in our understanding on the molecular neuropathology and pathobiology of TDP-43 in FTLD and ALS.
- Language
-
- English
- Open access status
- gold
- Identifiers
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- DOI 10.3390/ijms10010232
- PMID 19333444
- Persistent URL
- https://sonar.ch/global/documents/280241
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