… mice, and that the mutation triggers mRNA decay and, as a consequence, low … mRNA decay
as a therapeutic approach for individuals with progranulin-deficient frontotemporal dementia …

… Resultant transcripts are either targeted for nonsense-mediated decay due to a shift in the
open reading frame introducing a premature stop codon or are translated into novel protein …

… Most of the mutations are loss of function aberrations resulting in nonsense-mediated mRNA
decay. While GRN variants have been reported in a few ALS patients, these have not been …

… A common cause of frontotemporal dementia (FTD) are … mutations activate the
nonsense-mediated RNA decay (NMD) … in Grn R493X mRNA predicted to block its degradation by …

… and non-sense-mediated mRNA decay, increased levels of the … Psen1 mRNAs in KI/KI
brains is due to degradation of aberrantly spliced transcripts by non-sense-mediated mRNA decay…

… In the following we focus primarily on neurodegenerative disorders, where several NMD
targets have been implicated in ALS, and frontotemporal dementia (FTD) (depicted in Figure 3, …

… mRNA downregulation through two different ways: mRNA cleavage in case of perfect
complementarity between miRNA and target mRNA or … variants of frontotemporal dementia (FTD), …

… , we find that overall activity of nonsense-mediated mRNA decay (NMD), a key RNA quality-…
forms of frontotemporal dementia, collectively known as frontotemporal lobar dementia (FTLD…

… % of ALS patients also receive a diagnosis of frontotemporal dementia (FTD) and up to 50% …
third most common type of dementia after Alzheimer disease and Lewy body dementia [8]. …

… most common mutation associated with both frontotemporal dementia (FTD) and … degradation
of RNA from the expanded allele, there is likely to be some level of degradation of mRNA …