2016
DOI: 10.1080/19491034.2016.1172152
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Nucleocytoplasmic transport inC9orf72-mediated ALS/FTD

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Cited by 29 publications
(27 citation statements)
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“…R-DPRs inhibit NMD via translational repression. G 4 C 2 repeats and R-DPRs have also been associated with nucleocytoplasmic transport defects 33 . If nuclear export of RNA is delayed, both NMD targets and non-targets would be expected accumulate in the nucleus.…”
Section: Resultsmentioning
confidence: 99%
“…R-DPRs inhibit NMD via translational repression. G 4 C 2 repeats and R-DPRs have also been associated with nucleocytoplasmic transport defects 33 . If nuclear export of RNA is delayed, both NMD targets and non-targets would be expected accumulate in the nucleus.…”
Section: Resultsmentioning
confidence: 99%
“…The transport of mRNA and proteins between the nucleus and the cytoplasm has emerged as a critical cellular pathway in neurodegenerative diseases 24,28,30,61,62 . Here, we developed an approach to assess how the repeat expansion in C9orf72 impacts the subcellular N/C distribution of proteins.…”
Section: Discussionmentioning
confidence: 99%
“…Thus, it is possible that C9 DPR proteins may further impede mRNA transport throughout other areas of the cell via disruptions in nucleo/cytoplasmic transport. It is hypothesized that these defects may contribute to the mislocalization of key proteins such as TDP-43 early in the cascade of pathogenic events (Zhang K. et al, 2016 ). In turn, this could further impact RNA metabolism.…”
Section: C9orf72 Rna Based Mechanisms In Alsmentioning
confidence: 99%