Stress granule misprocessing is pathogenic in a mouse model of amyotrophic lateral sclerosis.

Takeaway

  • Stress granule misprocessing is involved in the pathogenesis of amyotrophic lateral sclerosis (ALS). The mouse model generated by the authors can be used to further investigate disease mechanisms.

Why this matters

  • Dysfunction of stress granule components, such as RNA-binding proteins FUS, TDP-43 and TIA1, causes amyotrophic lateral sclerosis (ALS). In FUS-mutant mice, oxidative stress was shown to aggravate motor performance decline and key hallmarks of disease pathology (mislocalization of stress granules and upregulation of ubiquitin).

  • The mouse model generated in this study is sound for further investigation in order to better understand the pathogenesis of ALS.