Mutant MATR3 (S85C) had been previously associated with an autosomal dominant distal myopathy with vocal cord and pharyngeal weakness (VCPDM) [5, 19]; however, in 2014, three different mutations (F115C, P154S, or T622A) in MATR3 were identified in cases of both fALS and sALS [7]. Here, MATR3 is linked to distal myopathy with vocal cord weakness.