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首页> 外文会议>Novartis Foundation symposium on Nuclear organization in development and disease >Skeletal and cardiac muscle defects in a murine model of Emery-Dreifuss muscular dystrophy
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Skeletal and cardiac muscle defects in a murine model of Emery-Dreifuss muscular dystrophy

机译：Emery-Dreifuss肌营养不良的小鼠模型中的骨骼和心肌缺陷

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Previous histological findings, physiological data, and behavioral observations on the A-type lamin knockout mouse (Lmna~l~) suggest that important aspects of this model resemble the human Emery-Dreifuss muscular dystrophy (EDMD) phenotype. The main goal of our experiments was to study skeletal and cardiac muscle function in this murine model to obtain the semiquantitative data needed for more detailed comparisons with human EDMD defects. Measurements of the mechanical properties of preparations from two different skeletal muscle groups, the sdleus and the diaphragm, were made in vitro. In addition, records of the electrocardiogram, and measurements of heart rate variability were obtained; and phasic contractions (unloaded shortening) of enzymatically isolated ventricular myocytes were monitored. Soleus muscles from Lwna~l~ mice produced less force and work than control preparations. In contrast, force and work production in strips of diaphragm were not changed significantly. Lead II electrocardiograms from conscious, restrained Lmna~i~ mice revealed slightly decreased heart rates, with significant prolongations of PQ, QRS, and 'QT' intervals compared with those from control recordings. These ECG changes resemble some aspects of the ECG records from humans with EDMD; however, the cardiac phenotype in this Lmna~l~ mouse model appears to be less well-defined/ developed. Ventricular myocytes isolated from Lmna~~ mice exhibited impaired contractile responses, particularly when superfused with the beta-adrenergic agonist, isoproterenol (1 uM). This deficit was more pronounced in myocytes isolated from the left ventride(s) than in myocytes from the right vcntricle(s). In summary, tissues from the L mna~l~ mouse exhibit a number of skeletal and cardiac muscle deficiencies, some of which are similar to those which have been reported in studies of human EDMD.
机译：先前的组织学发现，生理数据和对α型Lamin敲除小鼠（LMNA〜L〜）的行为观察表明该模型的重要方面类似于人砂肌肌肉营养不良（EDMD）表型。我们的实验的主要目的是研究该鼠模型中的骨骼和心肌功能，以获得与人EDMD缺陷更详细的比较所需的半定量数据。在体外制备来自两种不同骨骼肌组，Sdleus和隔膜的制剂的机械性能的测量。此外，获得了心电图的记录和心率变异性的测量;监测和酶促收缩（卸载缩短）被监测酶体肌细胞肌细胞。来自LWNA〜L〜小鼠的Soleus肌肉产生的力量和工作比控制制剂更少。相比之下，膜片条带的力量和工作生产不会显着变化。从有意识的II导心电图，内敛LMNA〜我只稍稍透露下降心脏速率，与PQ，QRS波显著的延长，以及“QT”间隔与对照相比录音。这些心电图变化类似于人类与EDMD的心电图记录的一些方面;然而，该LMNA〜L〜小鼠模型中的心脏表型似乎不太明确/开发。从LMNA ~~小鼠分离的心室肌细胞中表现出受损收缩反应，特别是当与β-肾上腺素能激动剂灌流，异丙基肾上腺素（1μM）停止。在左侧vcntricle的肌细胞中，肌细胞比来自右侧vcntricle的肌细胞更明显这种缺陷。总之，来自L MNA〜L〜小鼠的组织表现出许多骨骼和心肌缺陷，其中一些类似于人类EDMD研究中据报道的那些。

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