An R58W mutation in human patients causes defective repair in cultured muscle cells. (A) Representative images of FM1-43 infiltration in control or R58W human patient myocytes following injury. (B and C) FM1-43 accumulation time course following injury (B) and AUC values (C) for individual R58W or control myocytes. Control 1 n = 9 cells, Control 2 n = 8 cells, Patient n = 11 cells. (D) Images showing FM1-43 uptake in injured mouse ANO5-KO (KO), ANO5-KO + R58W ANO5 (KO+R58W), and WT fibers. (E and F) Kinetics of FM1-43 accumulation (E) and FM1-43 time course AUCs (F) from mouse myofibers with or without expression of R58W ANO5. ANO5-KO n = 22 fibers from four mice, ANO5-KO + R58W n = 31 fibers from four mice, WT n = 11 fibers from one mouse. (G) FM1-43 patch volumes obtained by integrating patch areas from individual slices (separated by 0.5 µm) of z-stacks acquired ∼10 min after injury. ANO5-KO n = 12 fibers from three mice, ANO5-KO + R58W n = 16 fibers from two mice. Data are mean ± SEM.