•PABP-1 is colocalized with mature TDP-43 inclusions, not with TDP-43 pre-inclusions.•PABP-1, colocalized with TDP-43 inclusions, in the ALS's with ATXN2 and C9orf72 mutations.•The frequency of PABP-1 colocalization with TDP-43 inclusions in ALS's without mutation is 36%, in ALS's with ATXN2 mutation is 47% and in ALS's with C9orf72 mutation is 67%.•In patients with FUS pathology, PABP-1 observed with pathologic FUS in the motor neurons. Here, ATXN2 is linked to amyotrophic lateral sclerosis.