•Ataxin-2 is present in the structure of SGs, which rapidly respond to stress and insults that affect the cell, and preventing translation of incorporate mRNA.•In controls, ataxin-2 was rarely observed in FUS inclusions Whereas in the ALS's, ataxin-2 was often seen with FUS cytoplasmic inclusions.•IP revealed that FUS was co-precipitated with ataxin-2 and not in controls.•Interaction between ataxin-2 and FUS is also RNA-independent, which was completed with RNase treatment. Here, FUS is linked to amyotrophic lateral sclerosis.