The ER under rapid fire

Amyotrophic lateral sclerosis ( ALS ) is a fatal neurodegenerative disease leading to selective death of upper and lower motoneurons. Clinically, the ALS syndrome is linked to pathogenic mutations in superoxide dismutase 1 (SOD1) , though actual molecular mechanisms remain ill understood. Two papers recently published in C ell S tem C ell and C ell R eports employ syngenic, iPSC ‐derived cell lines of one of the most severe SOD 1 mutations to report mitochondrial and ER stress as causal for perturbed electrical activity in ALS neurons (Kiskinis et al , [Kiskinis E, 2014]; Wainger et al , [Wainger BJ, 2014]).