TDP-43 proteinopathy in ALS is triggered by loss of ASRGL1 and associated with HML-2 expression.

Although the effect of ASRGL1 knockdown on the accumulation of TDP-43 was highly significant compared to the cells transfected with scrambled shRNA, it is difficult to compare it with the effect of MG132.

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In the sporadic and C9orf ALS groups, ASRGL1 expression was also reduced mostly in astrocytes (GFAP−), although it did not reach statistical significance (Supplementary Fig. 3 ).

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