Immune-mediated pathology in Duchenne muscular dystrophy.
review · Level V
Where this comes from
- Record sourced from PubMed, PMID 26246170.
- Also identified by DOI 10.1126/scitranslmed.aaa7322 and PMC identifier 5951380.
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Abstract
Immunological and inflammatory processes downstream of dystrophin deficiency as well as metabolic abnormalities, defective autophagy, and loss of regenerative capacity all contribute to muscle pathology in Duchenne muscular dystrophy (DMD). These downstream cascades offer potential avenues for pharmacological intervention. Modulating the inflammatory response and inducing immunological tolerance to de novo dystrophin expression will be critical to the success of dystrophin-replacement therapies. This Review focuses on the role of the inflammatory response in DMD pathogenesis and opportunities for clinical intervention.
Medical subject headings
- Immunity, Innate
- Muscular Dystrophy, Duchenne