Fig. 6: ASRGL1 rescues TDP-43-proteinopathy and associated neurotoxicity.
From: TDP-43 proteinopathy in ALS is triggered by loss of ASRGL1 and associated with HML-2 expression
a, b iPSCs-derived human neuronal cultures from a normal donor were co-transfected with TDP-43, with shRNAs against ASRGL1 or scrambled shRNAs and with increasing concentrations of an ASRGL1-encoding plasmid or with pcDNA as a control. The number of cells presenting cytoplasmic TDP-43 was analyzed by immunofluorescence followed by confocal microscopy. a Percentage of neurons presenting cytoplasmic TDP-43 (one-way ANOVA with Bonferroni correction; Mean ± SEM; number of experimental replicates = 4). b Percentage of neuronal viability in relation to the control (pcDNA), as measured by flour spectrometry after applying Alamarblue (Thermo Fisher) (Brown-Forsythe ANOVA test with Bonferroni correction; Mean ± SEM; number of experimental replicates = 6). c, d IPSc-derived human neuronal cultures from a normal donor were co-transfected with wild type or isoaspartate containing C-terminal TDP-43 peptides, and with an ASRGL1 plasmid or an empty vector (pcDNA). c Percentage of neuronal viability in relation to the control (neurons transfected with pcDNA) (Brown-Forsythe ANOVA test with Bonferroni correction; Mean ± SEM; number of experimental replicates = 6). d Neurite length expressed as a percentage of the control (neurons transfected with pcDNA) (Brown-Forsythe ANOVA test with Bonferroni correction; Mean ± SEM; number of experimental replicates = 6). All pairwise comparisons in the Figure were performed with two-sided tests. Source data are provided as a Source Data file.
