Journal
NEURON
Volume 96, Issue 2, Pages 285-297Publisher
CELL PRESS
DOI: 10.1016/j.neuron.2017.07.029
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Funding
- NIH [R35NS097974]
- Packard Center for ALS Research at the Johns Hopkins University [2002289810]
- ALS Association [16-IIP-270]
- Clinical Research in ALS and related disorders for Therapeutic Development (CReATe) Consortium [1U54NS092091]
- American-Lebanese-Syrian Associated Charities
- Howard Hughes Medical Institute [045104-Taylor]
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Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease characterized by degeneration of upper and lower motor neurons in the brain and spinal cord. The hallmark pathological feature in most cases of ALS is nuclear depletion and cytoplasmic accumulation of the protein TDP-43 in degenerating neurons. Consistent with this pattern of intracellular protein redistribution, impaired nucleocytoplasmic trafficking has emerged as a mechanism contributing to ALS pathology. Dysfunction in nucleocytoplasmic transport is also an emerging theme in physiological aging and other related neurodegenerative diseases, such as Huntington's and Alzheimer's diseases. Here we review transport through the nuclear pore complex, pointing out vulnerabilities that may underlie ALS and potentially contribute to this and other age-related neurodegenerative diseases.
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