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- Title
Nuclear transport dysfunction: a common theme in amyotrophic lateral sclerosis and frontotemporal dementia.
- Authors
Jovičić, Ana; Paul, Joseph W.; Gitler, Aaron D.
- Abstract
Amyotrophic lateral sclerosis ( ALS) and frontotemporal dementia ( FTD) are neurodegenerative diseases with overlapping genetic factors and pathology. On the cellular level, a majority of ALS and FTD cases are characterized by nuclear clearance and cytoplasmic aggregation of otherwise nuclear proteins, TAR DNA-binding protein 43 ( TDP-43), or fused in sarcoma. Recent studies investigating cellular pathways perturbed by genetic risk factors for ALS/ FTD converge on nucleocytoplasmic transport dysfunction as a mechanism leading to disease pathophysiology.
- Subjects
GENETICS of amyotrophic lateral sclerosis; FRONTOTEMPORAL dementia; NEURODEGENERATION; DNA-binding proteins; SARCOMA; GENETICS
- Publication
Journal of Neurochemistry, 2016, Vol 138, p134
- ISSN
0022-3042
- Publication type
Article
- DOI
10.1111/jnc.13642