40478_2020_1060_MOESM3_ESM.tif (19.82 MB)
Additional file 3 of RNA dependent suppression of C9orf72 ALS/FTD associated neurodegeneration by Matrin-3
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posted on 2020-11-01, 04:44 authored by Nandini Ramesh, Elizabeth L. Daley, Amanda M. Gleixner, Jacob R. Mann, Sukhleen Kour, Darilang Mawrie, Eric N. Anderson, Julia Kofler, Christopher J. Donnelly, Evangelos Kiskinis, Udai Bhan PandeyLevels of nuclear Histone H3 not altered in C9-ALS patient neurons. (A) Representative confocal images of C9-ALS iPSC differentiated to neurons (represented by MAP2, red) stained for Histone H3 nuclear protein (green/gray) (B) Quantification of endogenous Histone H3 immunofluorescence levels in MAP2+ neurons revealed no significant differences in the levels of nuclear Histone H3 in C9-ALS iPSC-MNs compared to that in control iPSC-MNs (Unpaired t test) n = 3 (C) Quantification of HIST1H3D mRNA fold change in control and C9-ALS iPSC MNs showed no significant differences in the mRNA levels in C9-ALS iPSC-MNs compared to that in control (Unpaired t-test) n = 10-12. Error bars indicate S.E.M
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G 4C neurodegenerationRANMatrin -3 AbstractiPSC-derived motor neuronsC 9orf ALSALS-associated RNA-binding proteinC 9 toxicityC 9-ALS patient-derived motor neuronsC 9orf mutationG 4C hexanucleotideintranuclear RNA fociC 9orf geneC 9-ALS patient-derived iPSC neuronsG 4C RNA fociMATR 3MATR 3. ImportantlyRBPMATR 3-mediated suppressionGGGGCC