The Changed Transcriptome of Muscular Dystrophy and Inflammatory Myopathy: Contributions of Non-Coding RNAs to Muscle Damage and Recovery

Abstract

In order to successfully recover from damage, skeletal muscle tissue requires proper activation of a tightly orchestrated repair program. Non-coding RNAs actively participate in this complex process of demolition and rebuilding. In this review, the contribution of dysregulated non-coding RNA expression to disease-associated pathological changes is explored in hereditary muscular dystrophies and idiopathic inflammatory myopathies. Disturbances in spatiotemporal expression of non-coding RNAs appear to be key factors in disease progression, functioning both in favor of and opposed to recovery. They regulate regeneration and survival of muscle fibers as well as codetermine the severity of tissue fibrosis and inflammation. Non-coding RNAs display individual or pleiotropic effects, and strongly influence each other’s activities. The described altered expression patterns can be exploited as biomarkers for diagnosis and to evaluate therapeutic success. In addition, common signatures of these non-coding RNAs often present in different muscle disorders point to their manipulation as an approach for potentially broader therapeutic use.