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  Indian J Med Microbiol
 

Figure 1: Three severe and two mild ALS8 patients from the same family, all carrying the same VAPB P56S genetic mutation were studied along with three controls. PBMCs were separately collected from these subjects, and then reprogrammed into induced pluripotent stem cells (iPSCs). iPSCs were differentiated into motor neurons, which were found to be dissimilar between the two ALS8 groups in terms of cell viability, mitochondrial activity, energetic metabolism and levels of expression of genes involved in protein synthesis; increased activity of such pathways in the motor neurons of mild ALS8 patients compared with the severe ones suggests mitigating mechanisms of neurodegeneration.

Figure 1: Three severe and two mild ALS8 patients from the same family, all carrying the same VAPB P56S genetic mutation were studied along with three controls.
PBMCs were separately collected from these subjects, and then reprogrammed into induced pluripotent stem cells (iPSCs). iPSCs were differentiated into motor neurons, which were found to be dissimilar between the two ALS8 groups in terms of cell viability, mitochondrial activity, energetic metabolism and levels of expression of genes involved in protein synthesis; increased activity of such pathways in the motor neurons of mild ALS8 patients compared with the severe ones suggests mitigating mechanisms of neurodegeneration.