Fig. 1.

Burrowing dys-1 mutants recapitulate cellular, behavioral, and life span phenotypes associated with DMD. (A–D) Representative TEM images. (A) WT animals that burrowed in 6% agar for 5 d displayed healthy body-wall muscles with well-ordered thin and thick filaments, clear dense bodies, and rounded mitochondria. (B) After 5 d, dystrophic animals lost muscle fiber organization, including blebbing mitochondria and rounding of the muscle cells. (C) In control burrowing animals, mitochondria displayed clear cristae (white arrowheads) and roundness. (D) Dystrophic animals possessed less round mitochondria that displayed signs of membrane degradation (red arrows). (E and F) Representative 3D structured illumination microscopy superresolution images. (E) Fluorescence-tagged mitochondria lines in crawling dys-1(eg33) mutants, which maintained clearly visible lines of mitochondria along the muscle fibers. (F) Burrowing dystrophic animals displayed blebbing mitochondria lines and increased circularity. On average, mitochondria were more circular (G), and smaller (H) in burrowing animals. (I) dys-1 mutants carrying independent loss-of-function alleles eg33 and cx18 showed decreased burrowing velocity. (J) Adult dys-1(eg33) worms cultivated in agar showed decreased longevity. Refer to Dataset S1 or results section for comparative and descriptive statistics. **P < 0.001, *P < 0.05.