1. Muscle contractures in patients with cerebral palsy and acquired brain injury are associated with extracellular matrix expansion, pro-inflammatory gene expression, and reduced rRNA synthesis.
- Author
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Von Walden F, Gantelius S, Liu C, Borgström H, Björk L, Gremark O, Stål P, Nader GA, and PontéN E
- Subjects
- Adolescent, Cell Count, Child, Collagen metabolism, Cytokines biosynthesis, Cytokines genetics, Female, Gene Expression Regulation, Humans, Male, Muscle Fibers, Skeletal pathology, RNA, Ribosomal genetics, Real-Time Polymerase Chain Reaction, Ribosomes genetics, Ribosomes pathology, Satellite Cells, Skeletal Muscle pathology, Brain Injuries pathology, Cerebral Palsy pathology, Extracellular Matrix pathology, Muscle, Skeletal pathology, RNA, Ribosomal biosynthesis
- Abstract
Introduction: Children with cerebral palsy (CP) and acquired brain injury (ABI) commonly develop muscle contractures with advancing age. An underlying growth defect contributing to skeletal muscle contracture formation in CP/ABI has been suggested., Methods: The biceps muscles of children and adolescents with CP/ABI (n = 20) and typically developing controls (n = 10) were investigated. We used immunohistochemistry, quantitative real-time polymerase chain reaction, and Western blotting to assess gene expression relevant to growth and size homeostasis., Results: Classical pro-inflammatory cytokines and genes involved in extracellular matrix (ECM) production were elevated in skeletal muscle of children with CP/ABI. Intramuscular collagen content was increased and satellite cell number decreased and this was associated with reduced levels of RNA polymerase I transcription factors, 45s pre-rRNA and 28S rRNA., Discussion: The present study provides novel data suggesting a role for pro-inflammatory cytokines and reduced ribosomal production in the development/maintenance of muscle contractures, possibly underlying stunted growth and perimysial ECM expansion. Muscle Nerve 58: 277-285, 2018., (© 2018 Wiley Periodicals, Inc.)
- Published
- 2018
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