Fort-Aznar, Laura; Ugbode, Chris; Sweeney, Sean T. published the artcile< Retrovirus reactivation in CHMP2BIntron5 models of frontotemporal dementia>, COA of Formula: C19H34O2, the main research area is frontotemporal dementia ALS retrovirus reactivation reverse transcriptase CHMP2B; Drosophila ; gypsy ; amyotrophic lateral sclerosis; frontotemporal dementia; retrovirus.
Frontotemporal dementia (FTD) is the second most prevalent form of pre-senile dementia after Alzheimer′s disease. Amyotrophic lateral sclerosis (ALS) can overlap genetically, pathol. and clin. with FTD indicating the two conditions are ends of a spectrum and may share common pathol. mechanisms. FTD-ALS causing mutations are known to be involved in endosomal trafficking and RNA regulation. Using an unbiased genome-wide genetic screen to identify mutations affecting an FTD-ALS-related phenotype in Drosophila caused by CHMP2BIntron5 expression, we have uncovered repressors of retrovirus (RV) activity as modifiers of CHMP2BIntron5 toxicity. We report that neuronal expression of CHMP2BIntron5 causes an increase in the activity of the endogenous Drosophila RV, gypsy, in the nervous system. Genetically blocking Drosophila gypsy activation and pharmacol. inhibiting viral reverse transcriptase activity prevents degenerative phenotypes observed in fly and rat neurons. These findings directly link endosomal dysfunction to RV de-repression in an FTD-ALS model without TDP-43 pathol. These observations may contribute an understanding to previous discoveries of RV activation in ALS affected patients.
Human Molecular Genetics published new progress about Amyotrophic lateral sclerosis. 112-63-0 belongs to class esters-buliding-blocks, and the molecular formula is C19H34O2, COA of Formula: C19H34O2.
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