Marfan syndrome (SMF) is an autosomal dominant systemic disease that affects connective tissue due to mutations in the fibrillin-1 gene that cause alteration in fibrillin-1 protein, one of the major microfibril components of the extracellular matrix of connective tissue. Individuals with SMF have lower muscle mass characterized by reduced size and number of muscle fibers and consequent lower muscle strength and greater muscle fatigue, but the molecular mechanisms involved in these changes are not yet well understood. In this sense, it is possible to hypothesize that the IKK / NF-kB signaling, which is an inductor of muscle atrophy could mediate the reduction in muscle mass observed in SMF. Studies have shown that eccentric exercise is effective in preventing muscle loss and / or improving muscle strength; Therefore, this exercise can be aplied as a viable physiotherapy rehabilitation strategy for patients with SMF. Thus, this project aims to evaluate the effect of an eccentric exercise protocol (run downhill) on the modulation of NF-kB expression in skeletal muscles from a Marfan syndrome mouse model (please verify the details in the project).
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