iPSC NeuroHub

Haploinsufficiency leads to neurodegeneration in C9ORF72 ALS/FTD human induced motor neurons

March 19, 2018

In Nature Medicine Shi et al.  use human iPSCs to model amyotrophic lateral sclerosis. iPSCs were generated from patients with expanded repeats in C9ORF72 as well engineered iPSCs with one or both copies of the gene being deactivated. These iPSCs were differentiated to spinal motor neurons through an induction protocol. They found that motor neurons derived from both patient subjects and engineered lines has decreased survivabilty when challenged… Read more