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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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2018 (English)In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 58, no 2, p. 277-285Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
Wiley-Blackwell, 2018. Vol. 58, no 2, p. 277-285
Keywords [en]
cerebral palsy, cytokine, extracellular matrix, ribosome biogenesis, skeletal muscle
National Category
Physiology
Identifiers
URN: urn:nbn:se:umu:diva-152418DOI: 10.1002/mus.26130ISI: 000445090300023PubMedID: 29572878OAI: oai:DiVA.org:umu-152418DiVA, id: diva2:1253538
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Stockholm County CouncilAvailable from: 2018-10-05 Created: 2018-10-05 Last updated: 2018-10-05Bibliographically approved

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Stål, Per

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Von Walden, FerdinandStål, Per
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CiteExportLink to record
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