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. Issue 2 (24th May 2018)

• Record Type: Journal Article
• Authors: Von Walden, Ferdinand; Gantelius, Stefan; Liu, Chang; Borgström, Hanna; Björk, Lars; Gremark, Ola; Stål, Per; Nader, Gustavo A.; PontéN, Eva
• Is Part Of: Muscle & nerve. Volume 58:Issue 2(2018)
• Page Start: 277
• Page End: 285
• Publication Date: 2018-05-24
• Subjects: cerebral palsy -- cytokine -- extracellular matrix -- ribosome biogenesis -- skeletal muscle; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.26130
• Languages: English
• ISSNs: 0148-639X

Abstract

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