Nonuniform hip adductor muscle deficits in children with cerebral palsy: a comparison between the gracilis and adductor longus.

IF 5.4 2区 生物学 Q2 CELL BIOLOGY
Faizan Syed, Latif Omerkhil, Jason J Howard, Christopher Tiessen, Sunnie Vuong, Ruth-Anne Seerattan, Gavin K Thomas, Sach Dabgotra, Isaac Obrigewitsch, Shuyue Liu, Timothy R Leonard, Venus Joumaa, Walter Herzog
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Abstract

Cerebral palsy (CP) is the most common cause of childhood physical disability and often results in muscle weakness, spasticity, and contractures. Although cellular and sarcomeric impairments have been documented in muscles from children with CP, systematic comparisons of multiple muscles within the same child remain limited. Therefore, we investigated within-subject differences in single muscle fiber mechanics, sarcomeric protein content, and histological properties between patient-paired adductor longus (AL) and gracilis (GR) muscle biopsies obtained from eleven children with severe spastic CP. Maximal active stress and active elastic modulus were significantly lower in AL than GR for both type I and type II muscle fibers, with passive stress also being lower in AL fibers at longer sarcomere lengths (3.0-3.4 µm). AL samples had significantly lower actin and nebulin content compared with GR, whereas myosin and titin did not differ significantly. Fiber cross-sectional area (CSA) was significantly smaller in AL than GR, whereas collagen, fat content, and myosin heavy chain (MHC) isoform distribution did not differ between the two muscles. These findings suggest that muscles with overlapping function can undergo substantially different cellular adaptations within the same child with CP, and treating the hip adductors as a single functional unit may obscure notable muscle-specific adaptations.NEW & NOTEWORTHY To our knowledge, this is the first study to show that two hip adductor muscles from the same children with spastic cerebral palsy differ in single muscle fiber force production and thin-filament protein content. These findings suggest that cellular muscle properties in cerebral palsy can vary substantially between muscles within the same child, even among muscles commonly treated as one functional group.

脑瘫患儿髋内收肌不均匀缺损:股薄肌和长内收肌的比较。
脑瘫(CP)是儿童身体残疾的最常见原因,通常导致肌肉无力、痉挛和挛缩。虽然在患有CP的儿童的肌肉中有细胞和肌肉组织损伤的记录,但对同一儿童的多个肌肉的系统比较仍然有限。因此,我们研究了11例患有严重痉挛性脑瘫的儿童的患者对长内收肌(AL)和股薄肌(GR)肌肉活检在单个肌纤维力学、肌肉蛋白含量和组织学特性上的差异。I型和II型肌纤维的最大活动应力和活动弹性模量在AL组明显低于GR组。当肌节长度较长(3.0 - 3.4 μ m)时,AL纤维的被动应力也较低。与GR相比,AL样品的肌动蛋白和星云蛋白含量显著降低,而肌凝蛋白和肌凝蛋白含量差异不显著。AL肌肉的纤维横截面积(CSA)明显小于GR肌肉,而胶原蛋白、脂肪含量和MHC异构体分布在两种肌肉之间没有差异。这些发现表明,在同一个CP患儿中,具有重叠功能的肌肉可以经历本质上不同的细胞适应,并且将髋关节内收肌作为一个单一的功能单位可能会掩盖显著的肌肉特异性适应。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
9.10
自引率
1.80%
发文量
252
审稿时长
1 months
期刊介绍: The American Journal of Physiology-Cell Physiology is dedicated to innovative approaches to the study of cell and molecular physiology. Contributions that use cellular and molecular approaches to shed light on mechanisms of physiological control at higher levels of organization also appear regularly. Manuscripts dealing with the structure and function of cell membranes, contractile systems, cellular organelles, and membrane channels, transporters, and pumps are encouraged. Studies dealing with integrated regulation of cellular function, including mechanisms of signal transduction, development, gene expression, cell-to-cell interactions, and the cell physiology of pathophysiological states, are also eagerly sought. Interdisciplinary studies that apply the approaches of biochemistry, biophysics, molecular biology, morphology, and immunology to the determination of new principles in cell physiology are especially welcome.
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