Regulation of utrophin a expression in c2c12 cells through variations in 5 utr
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Abstract
Duchenne Muscular Dystrophy (DMD) is the most frequent genetic
newlinedisorder that affects 1 in every 3500 males worldwide. The cause of the
newlinedisease is mutation in X-linked dystrophin gene. In absence of functional
newlinedystrophin the sarcolemmal integrity is lost resulting in muscle destruction
newlinethat leads to death. Dystrophin is one of the components of dystrophin
newlineassociated protein complex (DAPC) which linked the cytoskeleton with
newlineextracellular matrix. Utrophin is an autosomal homologue of dystrophin
newlinethat can compensate dystrophin deficiency. Expression of utrophin in
newlineadult is restricted to neuromuscular and myotendinous junctions. A two
newlinefold increase in expression of utrophin in mice model has been shown to
newlinereduce dystrophic pathology. Therefore upregulation of utrophin has been
newlineconsidered as a potential strategy for DMD therapy.
newline