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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

Von Walden, Ferdinand (author)
Karolinska Institutet
Gantelius, Stefan (author)
Karolinska Institutet
Liu, Chang (author)
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Borgström, Hanna (author)
Björk, Lars (author)
Karolinska Institutet
Gremark, Ola (author)
Stål, Per (author)
Umeå universitet,Institutionen för integrativ medicinsk biologi (IMB)
Nader, Gustavo A. (author)
Ponten, Eva (author)
Karolinska Institutet
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 (creator_code:org_t)
2018-05-24
2018
English.
In: Muscle and Nerve. - : Wiley-Blackwell. - 0148-639X .- 1097-4598. ; 58:2, s. 277-285
  • Journal article (peer-reviewed)
Abstract Subject headings
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  • 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.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Medicinska och farmaceutiska grundvetenskaper -- Fysiologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Basic Medicine -- Physiology (hsv//eng)

Keyword

cerebral palsy
cytokine
extracellular matrix
ribosome biogenesis
skeletal muscle

Publication and Content Type

ref (subject category)
art (subject category)

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