MATR3 and amyotrophic lateral sclerosis: Irrespective of the presence or absence of cytoplasmic matrin-3 aggregation, we also confirm the observation that nuclear matrin-3 staining is increased in sALS and fALS lumbar α-MNs.30 However, unlike mTDP-43 and mFUS, cytoplasmic accumulations of matrin-3 in fALS α-motor neurons are rare.30 Taken together, our immunohistochemical data from human ALS autopsy cases are in line with the above described results obtained with E102Q-SigR1 over-expressing cultured cells.