Altered localization of staufen1 in amyotrophic lateral sclerosis

Abstract

Localized protein expression is crucial for the health and survival of axons and dendrites in a rapidly changing environment. This process, however, cannot take place without the precise spatiotemporal localization of the cellular translational machinery and of mRNA. mRNA transport and localization requires a variety of RNA-binding proteins. Here, we highlight a recent publication which presents evidence for the altered localization of the dsRNA-binding protein Staufen1 as a result of Amyotrophic Lateral Sclerosis (ALS) linked mutations, supporting the perception of ALS as a RNA spatiotemporal mislocalization disease.