Ferdinand Von Walden1, Stefan Gantelius1,2, Chang Liu1, Hanna Borgström1, Lars Björk1, Ola Gremark3, Per Stål4, Gustavo A Nader5, Eva PontéN1,2. 1. Department of Women's and Children's health, Division of Neurology/Pediatric Orthopedics, Karolinska Institute, ALB Q2:07, Karolinska University Hospital, 17176, Stockholm, Sweden. 2. Department of Pediatric Orthopedic Surgery, Karolinska University Hospital, Stockholm, Sweden. 3. Department of Orthopedic Surgery, Danderyd Hospital, Stockholm, Sweden. 4. Department of Integrative Medical Biology, Laboratory of Muscle Biology, Umeå University, Sweden. 5. Department. of Kinesiology and Huck Institute of the Life Sciences, The Pennsylvania State University, University Park, Pennsylvania, USA.
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.
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.
Authors: Guido Weide; Peter A Huijing; Lynn Bar-On; Lizeth Sloot; Annemieke I Buizer; Jules G Becher; Jaap Harlaar; Richard T Jaspers Journal: Front Physiol Date: 2020-11-23 Impact factor: 4.566