Muscle phenotype of a rat model of Duchenne muscular dystrophy.

Muscle phenotype of a rat model of Duchenne muscular dystrophy.
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DOI:
10.1002/mus.27061
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发表时间:
2020-12
期刊:
影响因子:
3.4
通讯作者:
Lovering RM
Lovering RM
中科院分区:
医学3区
文献类型:
--
作者:
Iyer SR;Xu S;Shah SB;Lovering RM

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我们的目的是评估杜氏肌营养不良 (DMD) 大鼠模型 (Dmd-KO 大鼠) 的关键肌肉成像和收缩性参数,这些参数尚未得到充分表征。我们对大腿和腿部肌肉进行了体内磁共振成像 (MRI),并对特定后肢肌肉进行了苏木精和伊红 (H&E) 染色和体内肌肉收缩性测试。测试肌肉收缩力之前的 MRI 显示 Dmd-KO 大鼠股四头肌和胫骨前肌存在多个不均匀分布的局灶性高信号。 H&E 染色显示相应的炎症区域和正在进行的再生。体内收缩测试显示,与野生型 (WT) 大鼠相比,Dmd-KO 大鼠肌肉产生的最大力量显着降低,且对损伤的敏感性增加约两倍。总之,MRI 结果、组织学结果以及肌肉的低强度和高损伤敏感性支持使用 Dmd-KO 大鼠作为 DMD 动物模型。
Our aim was to assess key muscle imaging and contractility parameters in the Duchenne muscular dystrophy (DMD) rat model (Dmd-KO rat), which have not yet been characterized sufficiently. We performed in-vivo magnetic resonance imaging (MRI) for thigh and leg muscles, and performed hematoxylin and eosin (H&E) staining and in-vivo muscle contractility testing in specific hindlimb muscles. MRI prior to testing muscle contractility revealed multiple, unevenly distributed focal hyperintensities in the Dmd-KO rat quadriceps and tibialis anterior muscles. H&E staining showed corresponding areas of inflammation and ongoing regeneration. In-vivo contractile testing showed maximal force generated by Dmd-KO muscles was significantly lower, and susceptibility to injury was ~ two-fold greater in the Dmd-KO rats compared to wild-type (WT) rats. Together, the MRI findings, histological findings, and the low strength and high susceptibility to injury in muscles support use of the Dmd-KO rat as an animal model of DMD.
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