Energy metabolism has been repeatedly linked to amyotrophic lateral sclerosis (ALS). Yet,
motor neuron (MN) metabolism remains poorly studied and it is unknown if ALS MNs differ
metabolically from healthy MNs. To address this question, we first performed a metabolic
characterization of induced pluripotent stem cells (iPSCs) versus iPSC-derived MNs and
subsequently compared MNs from ALS patients carrying FUS mutations to their CRISPR/
Cas9-corrected counterparts.
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