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The Role of NF-κB in Muscular Dystrophy

Volume 4, Issue 2, 2013, pp. 149-160
DOI: 10.1615/ForumImmunDisTher.2013008386
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ABSTRACT

Aberrant activation of the nuclear factor−kappa B (NF-κB) pathway is associated with the fatal genetic muscle disease, Duchenne muscular dystrophy (DMD). NF-κB has been identified as a key player in the negative effects of chronic inflammation, which characterizes dystrophic skeletal muscle. This includes the suppression of myogenesis, which leads to decreased muscle regeneration and the exacerbation of muscular dystrophy. Several hypotheses have been proposed to explain the upregulation of NF-κB activity, and although differing significantly, they all point to the classical activation pathway, regulated by the inhibitor of kappa-B kinase (IKK). The dystrophic phenotype has been found to be significantly improved in transgenic dystrophic (mdx) mice with lowered NF-κB activity. Furthermore, several pharmacologic strategies of NF-κB blockade have demonstrated efficacy in DMD animal models, suggesting that this pathway has the potential to be a therapeutic target in human DMD patients.

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