40478_2020_1060_MOESM2_ESM.tif (22.58 MB)
Additional file 2 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 PandeyG4C2 colocalization with punctate and diffuse MATR3 in C9-ALS iPSC MNs. (A) Representative confocal images of C9-ALS patient-derived iPSCs that were differentiated to neurons (represented by MAP2, gray) showing colocalization between G4C2 RNA foci (green) with MATR3 (red) in diffuse form (yellow arrows). Dotted-white boxes show G4C2 foci represented in the high-magnification panels on the right (B) The percentage distribution of MATR3 puncta that co-localize with G4C2 foci (blue) and are independent of G4C2 foci (red) in two independent C9-ALS iPSC MNs (C9-ALS #1 and C9-ALS #2) and the combined average
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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
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