2017
DOI: 10.3390/jfmk2040044
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Abstract: Duchenne muscular dystrophy (DMD) is an X-linked genetic disease in which the dystrophin gene is mutated, resulting in dysfunctional dystrophin protein. Without dystrophin, the dystrophin-glycoprotein complex (DGC) is unstable, leading to an increase in muscle damage. Moreover, the imbalance between muscle damage and repair leads to a chronic inflammatory response and an increase in the amount of fibrosis over time. The absence of dystrophin at the sarcolemma also delocalizes and downregulates nitric oxide syn… Show more

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Cited by 16 publications
(20 citation statements)
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“…In particular, the over-expression of the local isoform of IGF-1 in mdx muscles (mdx/mIGF-1), not only induced significant muscle hypertrophy but also stimulated the maturation of the myogenic program, further supporting the maintenance of the differentiated phenotype [145, 146]. On the other side, inducing the expression of elevated circulating levels of IL-6 in mdx mice (mdx/IL-6) we obtained a significant worsening of dystrophic muscles, generating a more severe mouse model of DMD pathology [128, 147-149]. Although IL-6 is a positive regulator of SCs under physiologic conditions, its over-production dramatically impaired muscle differentiation in vitro and induced a progressive depletion of the SC pool in mdx mice at later stages of the pathology.…”
Section: Intrinsic and Extrinsic Alterations Of Stem Cell Biology In mentioning
confidence: 89%
“…In particular, the over-expression of the local isoform of IGF-1 in mdx muscles (mdx/mIGF-1), not only induced significant muscle hypertrophy but also stimulated the maturation of the myogenic program, further supporting the maintenance of the differentiated phenotype [145, 146]. On the other side, inducing the expression of elevated circulating levels of IL-6 in mdx mice (mdx/IL-6) we obtained a significant worsening of dystrophic muscles, generating a more severe mouse model of DMD pathology [128, 147-149]. Although IL-6 is a positive regulator of SCs under physiologic conditions, its over-production dramatically impaired muscle differentiation in vitro and induced a progressive depletion of the SC pool in mdx mice at later stages of the pathology.…”
Section: Intrinsic and Extrinsic Alterations Of Stem Cell Biology In mentioning
confidence: 89%
“…Various studies have investigated the mechanisms underlying skeletal muscle fibrosis and have identified several factors that affect fibrosis, such as oxidative stress, inflammation, and aging [ 1 , 6 , 7 ]. In particular, oxidative stress in DMD, which is mainly caused by dystrophin deficiency, is considered to be one of the major causes of muscle fibrosis [ 8 , 9 ]. Therefore, alleviation of oxidative stress-induced fibrosis is important in relieving the pathologic condition in patients with DMD.…”
Section: Introductionmentioning
confidence: 99%
“…The urgency to seek an effective treatment for DMD has resulted in the development of genetic and pharmacological interventions to correct or compensate for dystrophin deficiency, such as exon skipping (13,14), stop codon readthrough (15) and dystrophin gene therapies (16). Approaches to mitigate secondary and downstream pathological mechanisms (17,18) in parallel with translational efforts to define more accurate biomarkers and endpoints (19,20) have been also undertaken. Over the past two decades, more than 200 clinical trials in DMD patients have been conducted, are ongoing or are recruiting.…”
Section: Introductionmentioning
confidence: 99%