Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 22, Pages -Publisher
MDPI
DOI: 10.3390/ijms222212175
Keywords
neurodegeneration; motor neuron; ALS
Funding
- VIB
- KU Leuven
- Fund for Scientific Research Flanders (FWO-Vlaanderen)
- Agency for Innovation by Science and Technology (IWT)
- Agency for Innovation by Science and Technology (SBO-iPSCAF)
- Thierry Latran Foundation
- Muscular Dys-trophy Association (MDA)
- ALS Association (ALSA)
- Association Francaise contre les Myopathies (AFM)
- Association Belge contre les Maladies neuro-Musculaires (ABMM)
- Target ALS
- ALS Liga Belgie
- FWO-Vlaanderen
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There is evidence suggesting that nucleocytoplasmic-transport deficits may be important in the pathogenesis of ALS, but current data are limited in clarifying the exact causal and temporal role. Understanding this will be significant for therapeutically targeting nucleocytoplasmic transport and associated proteins.
There is ample evidence that nucleocytoplasmic-transport deficits could play an important role in the pathology of amyotrophic lateral sclerosis (ALS). However, the currently available data are often circumstantial and do not fully clarify the exact causal and temporal role of nucleocytoplasmic transport deficits in ALS patients. Gaining this knowledge will be of great significance in order to be able to target therapeutically nucleocytoplasmic transport and/or the proteins involved in this process. The availability of good model systems to study the nucleocytoplasmic transport process in detail will be especially crucial in investigating the effect of different mutations, as well as of other forms of stress. In this review, we discuss the evidence for the involvement of nucleocytoplasmic transport defects in ALS and the methods used to obtain these data. In addition, we provide an overview of the therapeutic strategies which could potentially counteract these defects.
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