期刊
NEUROSIGNALS
卷 16, 期 1, 页码 41-51出版社
KARGER
DOI: 10.1159/000109758
关键词
TDP-43; frontotemporal lobar degeneration; amyotrophic lateral sclerosis; neurodegenerative disease; frontotemporal dementia
资金
- NIA NIH HHS [AG17586, AG10124] Funding Source: Medline
- NATIONAL INSTITUTE ON AGING [P01AG017586, P30AG010124] Funding Source: NIH RePORTER
In this review, we summarize recent advances in understanding frontotemporal lobar degeneration (FTLD), amyotrophic lateral sclerosis (ALS) and related neurodegenerative disorders that are collectively known as TDP-43 proteinopathies, since transactive response DNA-binding protein 43 (TDP-43) was recently shown to be the major component of the ubiquitinated inclusions that are their pathological hallmarks. TDP-43 proteinopathies are distinct from most other neurodegenerative disorders because TDP-43 inclusions are not amyloid deposits. Besides TDP-43-positive inclusions, both sporadic and familial forms of FTLD and ALS have the pathologic TDP-43 signature of abnormal hyperphosphorylation, ubiquitination and C-terminal fragments in affected brain and spinal cord, suggesting that they share a common mechanism of pathogenesis. Thus, these findings support the concept that FTLD and ALS represent a clinicopathologic spectrum of one disease, that is, TDP-43 proteinopathy. Copyright (c) 2008 S. Karger AG, Basel.
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