In amyotrophic lateral sclerosis (ALS), some motor neuron types are more vulnerable to disease pathology. Here the authors show that resistant subtypes express the ER chaperone SIL1. Disease-associated loss of SIL1 impairs ER homeostasis and worsens ALS pathology, whereas its expression improves pathology and survival in an ALS mouse model.
- Audrey Filézac de L'Etang
- Niran Maharjan
- Smita Saxena
