Chronic Inflammation, Fibrotic Remodeling, and Regenerative Failure in Duchenne Muscular Dystrophy: Mechanisms and Therapeutic Opportunities
Duchenne muscular dystrophy (DMD) is an X-linked disorder initiated by dystrophin deficiency, but disease progression reflects more than sarcolemmal fragility. Recurrent myofiber injury sustains sterile inflammation through damage-associated innate immune signaling and downstream pathways including NF-κB and inflammasome activation. Persistent inflammation alters macrophage and fibro-adipogenic progenitor (FAP) behavior, promotes extracellular matrix remodeling, and creates a fibrotic niche that progressively limits effective repair. Regeneration is further compromised by both intrinsic muscle stem cell dysfunction and extrinsic constraints imposed by the remodeled microenvironment. This review integrates mechanistic and translational evidence linking these processes, with particular attention to FAP-centered stromal remodeling, immune–stem cell crosstalk, and tissue-specific differences between skeletal and cardiac muscle. We also evaluate emerging interventions targeting inflammatory priming, inflammasome activity, fibrotic remodeling, and regenerative competence while distinguishing established pathological mechanisms from predominantly preclinical therapeutic evidence. This perspective may help define stage-specific and niche-directed strategies that complement dystrophin restoration.
Authors
- Jie Shi (ORCID: https://orcid.org/0000-0002-7865-0113)
- Yang Jiao (ORCID: https://orcid.org/0000-0001-8476-7978)
Institutions
- First Affiliated Hospital of Xi'an Jiaotong University (CN)
- Lanzhou University Second Hospital (CN)
- Lanzhou University (CN)
- Xi'an Jiaotong University (CN)
Publication Details
- Journal
- Biomedicines
- Published
- 2026-09-28
- DOI
- https://doi.org/10.3390/biomedicines14102192
- Primary Topic
- Muscle Physiology and Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00