40478_2020_1060_MOESM1_ESM.tif (19.83 MB)
Additional file 1 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 PandeyControl iPSC-derived motor neurons and post-mortem brain tissues are negative for G4C2 foci. (A) Representative confocal images of control iPSC-MN indicated by MAP2 (gray). White dotted box represents area of high magnification in the images on the right. FISH-IF staining of G4C2 RNA foci (green) and MATR3 (red) showed no signal for G4C2 foci in control iPSC neurons, and thus no colocalization between MATR3 puncta (yellow arrow) and G4C2. (B) FISH-IF of control post-mortem brains are negative for G4C2 foci. White-dotted circle demarcates the nucleus. Asterisks denote autofluorescence due to lipofuscin
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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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