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.

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Publication Details

Journal
Biomedicines
Published
2026-09-28
DOI
https://doi.org/10.3390/biomedicines14102192
Primary Topic
Muscle Physiology and Disorders
Type
article
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article

Chronic Inflammation, Fibrotic Remodeling, and Regenerative Failure in Duchenne Muscular Dystrophy: Mechanisms and Therapeutic Opportunities

Jie Shi, Yang Jiao
Biomedicines
Muscle Physiology and Disorders
article

Chronic Inflammation, Fibrotic Remodeling, and Regenerative Failure in Duchenne Muscular Dystrophy: Mechanisms and Therapeutic Opportunities

Jie Shi, Yang Jiao
article en

Abstract

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.

BiomedicinesVol. 14(10)
First Affiliated Hospital of Xi'an Jiaotong University (CN), Lanzhou University Second Hospital (CN), Lanzhou University (CN), Xi'an Jiaotong University (CN)
Life in Land
Openalex Percentile: Top 20%
Muscle Physiology and Disorders
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