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Differential histological features and myogenic protein levels in distinct muscles of d-sarcoglycan null muscular dystrophy mouse model
Journal of Molecular Histology ( IF 3.2 ) Pub Date : 2023-06-26 , DOI: 10.1007/s10735-023-10136-7
Carlos Palma-Flores 1 , Luis Javier Cano-Martínez 2 , Francisca Fernández-Valverde 3 , Itzel Torres-Pérez 4 , Sergio de Los Santos 5 , J Manuel Hernández-Hernández 6 , Adriana Fabiola Hernández-Herrera 4 , Silvia García 4 , Patricia Canto 5 , Alejandro Zentella-Dehesa 7, 8 , Ramón Mauricio Coral-Vázquez 4, 9
Affiliation  

Skeletal muscle (SkM) comprises slow and fast-twitch fibers, which differ in molecular composition, function, and systemic energy consumption. In addition, muscular dystrophies (DM), a group of diverse hereditary diseases, present different patterns of muscle involvement, progression, and severity, suggesting that the regeneration-degeneration process may differ depending on the muscle type. Therefore, the study aimed to explore the expression of proteins involved in the repair process in different muscles at an early stage of muscular dystrophy in the δ-sarcoglycan null mice (Sgcd-null), a limb-girdle muscular dystrophy 2 F model. Hematoxylin & Eosin (H&E) Staining showed a high number of central nuclei in soleus (Sol), tibialis (Ta), gastrocnemius (Gas), and extensor digitorum longus (Edl) from four months Sgcd-null mice. However, fibrosis, determined by trichrome of Gomori modified staining, was only observed in Sgcd-null Sol. In addition, the number of Type I and II fibers variated differentially in the Sgcd-null muscles vs. wild-type muscles. Besides, the protein expression level of β-catenin, myomaker, MyoD, and myogenin also presented different expression levels in all the Sgcd-null muscles studied. In summary, our study reveals that muscles with different metabolic characteristics showed distinct expression patterns of proteins involved in the muscle regeneration process. These results could be relevant in designing therapies for genetic and acquired myopathy.



中文翻译:

d-肌聚糖无效肌营养不良症小鼠模型不同肌肉的差异组织学特征和肌源性蛋白水平

骨骼肌 (SkM) 由慢肌纤维和快肌纤维组成,它们的分子组成、功能和全身能量消耗不同。此外,肌营养不良症(DM)是一组不同的遗传性疾病,呈现出不同的肌肉受累模式、进展和严重程度,这表明再生-退化过程可能因肌肉类型而异。因此,本研究旨在探讨肢带型肌营养不良症2 F模型δ-肌聚糖无效小鼠( Sgcd -null)中肌营养不良症早期不同肌肉中参与修复过程的蛋白质的表达。苏木精和伊红 (H&E) 染色显示,四个月龄 Sgcd 缺失小鼠的比目鱼肌 (Sol)、胫骨肌 (Ta)、腓肠肌 (Gas) 和趾长伸肌 (Edl) 中存在大量中央核。然而,通过 Gomori 改良染色三色确定的纤维化仅在Sgcd -null Sol 中观察到。此外,Sgcd -null 肌肉与野生型肌肉中 I 型和 II 型纤维的数量存在差异。此外,β-catenin、myomaker、MyoD 和 myogenin 的蛋白表达水平在所有研究的Sgcd缺失肌肉中也呈现出不同的表达水平。总之,我们的研究表明,具有不同代谢特征的肌肉在肌肉再生过程中表现出不同的蛋白质表达模式。这些结果可能与设计遗传性和获得性肌病的疗法相关。

更新日期:2023-06-28
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