https://www.socictopen.socict.org/files/original/1c417185cdb95d1eb37d5e912898fdd0.pdf afd30191d4e6e48dedc189ba42393674 Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Coronavirus Description An account of the resource Dominio científico: Coronavirus Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource RNA pathogenesis via Toll-like receptor-activated inflammation in expanded repeat neurodegenerative diseases Creator An entity primarily responsible for making the resource Robert Ian Richards, Saumya E Samaraweera, Clare evan Eyk, Louise Veronica O'Keefe, Catherine eSuter Description An account of the resource Repeat sequences that are expanded in copy number are the basis for ~20 dominantly inherited neurodegenerative diseases, including Huntington’s Disease. Despite some of the responsible genes being identified as long as 20 years ago, the identity and nature of the disease-causing pathogenic pathway remains a gap in knowledge for these diseases. This understanding is essential for rational approaches to delay onset, slow progression or ultimately effect cure. We have previously hypothesized that an RNA-based pathogenic pathway has a causal role in the dominantly inherited unstable expanded repeat neurodegenerative diseases. In support of this hypothesis we, and others, have characterized rCAG.rCUG100 repeat double-strand RNA (dsRNA) as a previously unidentified agent capable of causing pathogenesis in a Drosophila model of neurodegenerative disease. Dicer, Toll and autophagy pathways have distinct roles in this Drosophila dsRNA pathology. Dicer-dependence is accompanied by cleavage of rCAG.rCUG100 repeat double-strand RNA down to r(CAG)7 21-mers. Among the ‘molecular hallmarks’ of this pathway that have been identified in Drosophila, some [i.e. r(CAG)7 and elevated TNF] correlate with observations in affected people (e.g. HD, ALS) or in related animal models [i.e. autophagy]. The Toll pathway is activated in the presence of repeat-containing double-stranded RNA and toxicity is also dependent on this pathway. How might the endogenously expressed dsRNA mediate Toll-dependent toxicity in neuronal cells? Endogenous RNAs are normally shielded from Toll pathway activation as part of the mechanism to distinguish ‘self’ from ‘non-self’ RNAs. This typically involves post-transcriptional modification of the RNA. Therefore, it is likely that rCAG.rCUG100 repeat double-strand RNA has a characteristic property that interferes with or evades this normal mechanism of shielding. We predict that repeat expansion leads to an alteration in RNA structure and/or form that perturbs RNA mod Date A point or period of time associated with an event in the lifecycle of the resource 2013 Subject The topic of the resource neurodegeneration, toll-like receptor, RNA pathogenesis, innate inflammation, expanded repeat diseases Identifier An unambiguous reference to the resource within a given context DOI: 10.3389/fnmol.2013.00025 Source A related resource from which the described resource is derived Frontiers in Molecular Neuroscience Publisher An entity responsible for making the resource available Frontiers Media S.A. Coverage The spatial or temporal topic of the resource, the spatial applicability of the resource, or the jurisdiction under which the resource is relevant Neurosciences. Biological psychiatry. Neuropsychiatry Language A language of the resource EN