Dystrophin-deficient myofibers are vulnerable to mast cell granule-induced necrosis

JRM Gorospe, M Tharp, T Demitsu… - Neuromuscular Disorders, 1994 - Elsevier
Duchenne muscular dystrophy is the most common inherited lethal X-linked disorder of
mankind and is caused by dystrophin deficiency. The steps involved in the dystrophin-
deficiency-induced cascade which lead to myofiber necrosis, progressive muscle wasting in
humans and dogs and prominent muscle hypertrophy in mice and cats are obscure.
Dystrophin is an intracellular component of the membrane cytoskeleton and its absence
would be expected to cause necrosis of isolated myofibers (cell autonomous defect) …