Fig. 2

Severe phenotypic MATR3^F115C transgenic mice develop profound motor phenotype and show gross muscle atrophy. Escape extension of mice at ~ 2 months showed no differences comparing a NT to b MATR3^WT and c MATR3^F115C (lines noted on figure). Escape extension of mice at ~ 10 months showed no difference comparing d NT to e MATR3^WT displaying no obvious motor phenotype, and f MATR3^F115C displaying a mild-to-moderate phenotype. A subset of ~ 10-month old g MATR3^F115C mice lacked the normal escape reflex. Gross hindlimb muscle showed no difference between ~ 10 month old h NT and i MATR3^WT; however, muscles appeared grossly atrophied in j MATR3^F115C displaying either a mild-to-moderate or k severe motor phenotype. Panels a-k are from female mice