@article{626e5b12ec6a4bbc81dfbe646f50141a,
title = "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",
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.",
author = "{Von Walden}, Ferdinand and Stefan Gantelius and Chang Liu and Hanna Borgstr{\"o}m and Lars Bj{\"o}rk and Ola Gremark and Per St{\aa}l and Nader, {Gustavo A.} and Eva Pont{\'e}N",
note = "Funding Information: Abbreviations: ABI, acquired brain injury; COL1A, collagen 1; CON, typically developing children; CP, cerebral palsy; CTGF, connective tissue growth factor; ECM, extracellular matrix; FBXO32, F-box protein 32/atro-gin-1; GAPDH, glyceralde-hyde-3-phosphate dehydrogenase; IL, interleukin; LOX, lysyl oxidase; LTBP1, latent transforming growth factor binding protein 1; MSTN, myostatin; POL, RNA polymerase; qRT-PCR, quantitative real-time polymerase chain reaction; rDNA, ribosomal DNA; ROI, region of interest; RP, ribosomal protein; rRNA, ribosomal RNA; SDS, sodium dodecyl sulfate; TGF-beta, transforming growth factor beta; TGFB1, transforming growth factor beta 1; TGFBR2, transforming growth factor beta receptor 2; TIF-1A, transcription initiation factor 1A; TNF, tumor necrosis factor; Trim32, tripartite motif containing 32; UBF, upstream binding factor Key words: cerebral palsy; cytokine; extracellular matrix; ribosome biogenesis; skeletal muscle Funding: The research has been performed with support from Stiftelsen Promobilia, Stiftelsen Sunnerdahls Handikappfond, Norrbacka-Eugenia-stiftelsen, Stiftelsen Samariten, Linnea och Josef Carlssons Stiftelse, and grants provided by the Stockholm County Council (ALF-project). Data generated in the Nader lab were supported by the Pennsylvania State University and Huck Institutes of the Life Sciences. Publisher Copyright: {\textcopyright} 2018 Wiley Periodicals, Inc.",
year = "2018",
month = aug,
doi = "10.1002/mus.26130",
language = "English (US)",
volume = "58",
pages = "277--285",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "2",
}