The Qualitative Effects of Resveratrol and Coenzyme Q10 Administration on the Gluteus Complex Muscle Morphology of SJL/J Mice with Dysferlinopathy Efectos Cualitativos de la Administraci車n de Resveratrol y Coenzima Q10 en la Morfolog赤a del Complejo Muscular Gl迆teo de Ratones SJL/J con Disferlinopat赤a

作者：Wendy Jeannette van der Spuy; Etheresia Pretorius

来源：International Journal of Morphology, 2011.

• Disferlinopatia
• Coenzima Q10
• Resveratrol
• Sarcolema
• Dysferlinopathy
• Coenzyme Q10
• Resveratrol
• Sarcolemma

Dysferlinopathy is a form of muscular dystrophy affecting muscles of the shoulder and pelvic girdles, resulting from inheritance of a mutated dysferlin gene. The encoded dysferlin protein is proposed to be involved in sarcolemmal vesicle fusion with a disrupted plasma membrane; however, with defective protein function these vesicles accumulate beneath the disruption site but are unable to fuse with it and reseal the membrane, thus rendering the membrane repair mechanism defective. The SJL/J mouse model presents with characteristics much like the commonest human condition. Immune modulators have long been under study in the maintenance of muscle health in muscular dystrophies. Such supplementary treatment would ideally suppress inflammation, preventing the immune response toward degenerating muscle from causing additional muscle fiber death, and thus provide a mechanism by which to prolong the life of muscle fibers with inherently defective healing apparatus. For this purpose the anti-inflammatory supplement resveratrol and the membrane-protective supplement coenzyme Q10 were administered separately and in combination to experimental animals to determine their effectiveness in possible therapy of dysferlinopathy. The findings of this study report that low doses of resveratrol and coenzyme Q10 supplementation in exclusivity were unable to afford much protection to muscle fibers at the tissue level. High doses of coenzyme Q10 proved more effective in reducing attenuating inflammation; and combination treatment with resveratrol and coenzyme Q10 provided not only the membrane-protective effects of coenzyme Q10, but also the anti-inflammatory effects of resveratrol which failed to materialize at sufficient levels in exclusive administration. Disferlinopat赤a es una forma de distrofia muscular que afecta a los m迆sculos de los hombros y cintura p谷lvica, resultado de la herencia y mutaci車n del gen de la distrofina.Sugerimos que la prote赤na codificada distrofina que integra la estructura sarcolemal con una membrana plasm芍tica interrumpida, que al presentar una prote赤na defectuosa, las estructuras se acumulan debajo del sitio de alteraci車n sin lograr fundirse con 谷ste y cerrar la membrana afectando el mecanismo de reparaci車n. El modelo de rat車n SJL / J se presenta con caracter赤sticas muy similares a una condici車n humana com迆n.Los inmunomoduladores han sido objeto de estudio en el mantenimiento de la salud muscular en las distrofias musculares.Este tipo de tratamiento suplementario puede ser ideal para suprimir la inflamaci車n, en la prevenci車n de la respuesta inmune