{"title":"Muscle satellite cells and fibro-adipogenic progenitors from muscle contractures of children with cerebral palsy have impaired regenerative capacity","authors":"","doi":"10.1111/dmcn.16063","DOIUrl":null,"url":null,"abstract":"<p>In cerebral palsy (CP), muscles often develop contractures, where the muscle is shorter and stiffer than healthy muscle. This impairs muscle function, limiting the ability of children with CP to have full range of motion and function of their muscles.</p><p>Muscles are made up of millions of cells and several different cell types. One of these key cell types form new muscle when the muscle is injured or growing. Another cell type produces a network of proteins that provide support and structure to the muscle. The goal of this study was to understand how these two cell types change with contracture. This will allow us to identify changes in cell behavior in CP and therefore identify targets to improve muscle function.</p><p>To do so, we compared cells taken from muscle biopsies of six children with CP and six typically developing (TD) children. We found that cells from children with CP did not form muscle as much as TD cells. These cells also had worse muscle formation on stiffnesses that represent contractures compared to healthy-like stiffnesses. This indicates that when the muscle is injured in CP, it cannot recover as easily. We found that treatment with a drug called verteporfin slightly improved muscle formation. We also found that the cells that produce the proteins and structure in muscle change into a more diseased state in CP compared to TD.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":null,"pages":null},"PeriodicalIF":3.8000,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16063","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Developmental Medicine and Child Neurology","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/dmcn.16063","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
In cerebral palsy (CP), muscles often develop contractures, where the muscle is shorter and stiffer than healthy muscle. This impairs muscle function, limiting the ability of children with CP to have full range of motion and function of their muscles.
Muscles are made up of millions of cells and several different cell types. One of these key cell types form new muscle when the muscle is injured or growing. Another cell type produces a network of proteins that provide support and structure to the muscle. The goal of this study was to understand how these two cell types change with contracture. This will allow us to identify changes in cell behavior in CP and therefore identify targets to improve muscle function.
To do so, we compared cells taken from muscle biopsies of six children with CP and six typically developing (TD) children. We found that cells from children with CP did not form muscle as much as TD cells. These cells also had worse muscle formation on stiffnesses that represent contractures compared to healthy-like stiffnesses. This indicates that when the muscle is injured in CP, it cannot recover as easily. We found that treatment with a drug called verteporfin slightly improved muscle formation. We also found that the cells that produce the proteins and structure in muscle change into a more diseased state in CP compared to TD.
期刊介绍:
Wiley-Blackwell is pleased to publish Developmental Medicine & Child Neurology (DMCN), a Mac Keith Press publication and official journal of the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) and the British Paediatric Neurology Association (BPNA).
For over 50 years, DMCN has defined the field of paediatric neurology and neurodisability and is one of the world’s leading journals in the whole field of paediatrics. DMCN disseminates a range of information worldwide to improve the lives of disabled children and their families. The high quality of published articles is maintained by expert review, including independent statistical assessment, before acceptance.