Inflammation in Duchenne Muscular Dystrophy-Exploring the Role of Neutrophils in Muscle Damage and Regeneration

被引:33
作者
Tulangekar, Ankita [1 ]
Sztal, Tamar E. [1 ]
机构
[1] Monash Univ, Sch Biol Sci, Melbourne, Vic 3800, Australia
基金
澳大利亚研究理事会;
关键词
Duchenne muscular dystrophy; DMD; inflammation; neutrophils; myeloperoxidase; neutrophil elastase; EXTRACELLULAR TRAPS; MACROPHAGE ACTIVATION; OXIDATIVE STRESS; SKELETAL; MYELOPEROXIDASE; PATHOGENESIS; INHIBITOR; FIBROSIS; NECROSIS; DISEASES;
D O I
10.3390/biomedicines9101366
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Duchenne muscular dystrophy (DMD) is a severe and progressive, X-linked, neuromuscular disorder caused by mutations in the dystrophin gene. In DMD, the lack of functional dystrophin protein makes the muscle membrane fragile, leaving the muscle fibers prone to damage during contraction. Muscle degeneration in DMD patients is closely associated with a prolonged inflammatory response, and while this is important to stimulate regeneration, inflammation is also thought to exacerbate muscle damage. Neutrophils are one of the first immune cells to be recruited to the damaged muscle and are the first line of defense during tissue injury or infection. Neutrophils can promote inflammation by releasing pro-inflammatory cytokines and compounds, including myeloperoxidase (MPO) and neutrophil elastase (NE), that lead to oxidative stress and are thought to have a role in prolonging inflammation in DMD. In this review, we provide an overview of the roles of the innate immune response, with particular focus on mechanisms used by neutrophils to exacerbate muscle damage and impair regeneration in DMD.</p>
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页数:11
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