… 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 …

… of frontotemporal dementia (FTD), primary progressive aphasia, semantic dementia,
Cortico-basal degeneration (… Frontotemporal dementia symptoms can also be present along with …

… Furthermore, sequestration of key RNA binding proteins may also play an important role in
… sclerosis and frontotemporal dementia linked genes into these two pathways highlights RNA …

… A common cause of frontotemporal dementia (FTD) are … RNA decay (NMD) pathway, we
sought to inhibit this RNA … in Grn R493X mRNA predicted to block its degradation by NMD. …

… Dysregulated RNA metabolism is emerging as a crucially important mechanism underpinning
the pathogenesis of frontotemporal dementia (FTD) and the clinically, genetically and …

… RNA-binding protein TDP43. Here, we show that TDP43 recognizes m6A RNA and that RNA
… We also observed extensive RNA hypermethylation in ALS spinal cord, corresponding to …

… on autophagy-mediated protein catabolism, accumulating data supports autophagy-mediated
RNA degradation as an important additional cellular RNA quality control mechanism. …

… 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 …

… Frontotemporal dementia is a group of early onset dementia syndromes linked to underlying
frontotemporal lobar degeneration … involving tau and two RNA binding proteins, TDP-43 and …

… what happens with targeted RNA degradation through use of siRNAs. This latter approach
reduces the nonpathogenic C9orf72 RNA without affecting nuclear RNA foci, consistent with …