当前位置: X-MOL 学术Connect. Tissue Res. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Contribution of extracellular matrix components to the stiffness of skeletal muscle contractures in patients with cerebral palsy
Connective Tissue Research ( IF 2.9 ) Pub Date : 2019-11-28 , DOI: 10.1080/03008207.2019.1694011
Lucas R Smith 1 , Rajeswari Pichika 2 , Rachel C Meza 3, 4 , Allison R Gillies 3 , Marwan N Baliki 2 , Henry G Chambers 5 , Richard L Lieber 2, 3, 6
Affiliation  

ABSTRACT

Purpose: Joint contractures in children with cerebral palsy contain muscle tissue that is mechanically stiffer with higher collagen content than typically developing children. Interestingly, the correlation between collagen content and stiffness is weak. To date, no data are available on collagen types or other extracellular matrix proteins in these muscles, nor any information regarding their function. Thus, our purpose was to measure specific extracellular protein composition in cerebral palsy and typically developing human muscles along with structural aspects of extracellular matrix architecture to determine the extent to which these explain mechanical properties. Materials and Methods: Biopsies were collected from children with cerebral palsy undergoing muscle lengthening procedures and typically developing children undergoing anterior cruciate ligament reconstruction. Tissue was prepared for the determination of collagen types I, III, IV, and VI, proteoglycan, biglycan, decorin, hyaluronic acid/uronic acid and collagen crosslinking. Results: All collagen types increased in cerebral palsy along with pyridinoline crosslinks, total proteoglycan, and uronic acid. In all cases, type I or total collagen and total proteoglycan were positive predictors, while biglycan was a negative predictor of stiffness. Together these parameters accounted for a greater degree of variance within groups than across groups, demonstrating an altered relationship between extracellular matrix and stiffness with cerebral palsy. Further, stereological analysis revealed a significant increase in collagen fibrils organized in cables and an increased volume fraction of fibroblasts in CP muscle. Conclusions: These data demonstrate a novel adaptation of muscle extracellular matrix in children with cerebral palsy that includes alterations in extracellular matrix protein composition and structure related to mechanical function.



中文翻译:

细胞外基质成分对脑瘫患者骨骼肌挛缩僵硬度的影响

摘要

目的: 脑瘫儿童的关节挛缩包含的肌肉组织比正常发育的儿童更坚硬,胶原蛋白含量更高。有趣的是,胶原蛋白含量和硬度之间的相关性很弱。迄今为止,没有关于这些肌肉中胶原蛋白类型或其他细胞外基质蛋白的数据,也没有关于它们功能的任何信息。因此,我们的目的是测量脑性瘫痪中特定的细胞外蛋白质组成,通常是人类肌肉发育以及细胞外基质结构的结构方面,以确定这些解释机械特性的程度。材料和方法: 从接受肌肉延长手术的脑瘫儿童和接受前交叉韧带重建的典型发育中的儿童身上采集活检样本。制备用于测定 I、III、IV 和 VI 型胶原蛋白、蛋白聚糖、双糖链蛋白聚糖、核心蛋白聚糖、透明质酸/糖醛酸和胶原交联的组织。结果:在脑瘫患者中,所有的胶原蛋白类型都随着吡啶啉交联、总蛋白多糖和糖醛酸的增加而增加。在所有情况下,I 型或总胶原蛋白和总蛋白多糖都是阳性预测因子,而双糖链蛋白聚糖是僵硬的阴性预测因子。这些参数共同解释了组内比组间更大程度的差异,表明细胞外基质和脑瘫僵硬之间的关系发生了改变。此外,立体学分析显示,在电缆中组织的胶原纤维显着增加,CP 肌肉中成纤维细胞的体积分数增加。结论:这些数据证明了脑瘫儿童肌肉细胞外基质的新适应,包括与机械功能相关的细胞外基质蛋白组成和结构的改变。

更新日期:2019-11-28
down
wechat
bug