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

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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
Funding Information
  • Stockholm County Council
  • Stiftelsen Promobilia
  • Stiftelsen Samariten
  • Pennsylvania State University
  • Stiftelsen Sunnerdahls Handikappfond
  • Huck Institutes of the Life Sciences
  • Norrbacka-Eugeniastiftelsen

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