A 2-year-old male patient develops progressive generalized weakness and muscle atrophy. The impairment first began with the muscles of the hips, and then progressed to the pelvic area, thigh, and shoulder muscles. The patient is diagnosed with Duchenne's muscular dystrophy, a congenital disorder where the protein dystrophin is deficient. Which of the following describes the role of dystrophin in muscle tissue?
- anchors actin to the sarcolemma
- endows the myosin filaments with elastic recoil properties
- extends from Z disk to Zdisk, forming a supportive network
- inhibits the binding of myosin to actin
- protects desmin filaments from stressinduced damage
Answer(s): A
Explanation:
Dystrophin anchors actin to the sarcolemma, reinforcing and stabilizing the latter during muscle contraction. Titin is a large protein which associates with myosin filaments and endows them with elastic recoil properties (choice B). Desmin filaments form a supportive network extending from Z disk to Zdisk (choice C). Troponin I inhibits the binding of myosin to actin (choice D). AlphaB-crystallin protects desmin filaments from stress-induced damage (choice E).
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