磁共振成像评估 δ-横纹肌聚糖缺失小鼠的心脏功能障碍。
Magnetic resonance imaging assessment of cardiac dysfunction in δ-sarcoglycan null mice.
机构信息
Department of Radiology/Imaging Research Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
出版信息
Neuromuscul Disord. 2011 Jan;21(1):68-73. doi: 10.1016/j.nmd.2010.09.007. Epub 2010 Oct 8.
Delta-sarcoglycan (δ-sarcoglycan) null, Scgd(-/-), mice develop cardiac and skeletal muscle histopathological alterations similar to those in humans with limb-girdle muscular dystrophy. The objective of this study was to assess the feasibility of using MRI to investigate cardiac dysfunction in Scgd(-/-) mice. Cardiac MRI of 8 month old Scgd(-/-) and wild type (WT) mice was performed. Compared to WT, Scgd(-/-) mice had significantly lower LV ejection fraction (44±5% vs. 66±4%, p=0.014), lower RV ejection fraction (25±2% vs. 51±3%, p<0.001) lower myocardial circumferential strain, (15.0±0.3% vs. 16.9±0.3%, p=0.007) and RV dilatation (54±3 μL vs. 40±3 μL, p=0.007). The regional circumferential strain also demonstrated significant temporal dyssynchrony between opposing regions of the Scgd(-/-) LV. Our results demonstrate severe cardiac dysfunction in Scgd(-/-) mice at 8 months. The study identifies a set of non-invasive markers that could be used to study efficacy of novel therapeutic agents in dystrophic mice.
δ-横纹肌聚糖（δ-sarcoglycan）缺失型（Scgd(-/-)）小鼠表现出与肢带型肌营养不良症人类患者相似的心脏和骨骼肌组织病理学改变。本研究旨在评估 MRI 用于研究 Scgd(-/-) 小鼠心脏功能障碍的可行性。对 8 月龄 Scgd(-/-)和野生型（WT）小鼠进行心脏 MRI 检查。与 WT 相比，Scgd(-/-) 小鼠的左心室射血分数明显降低（44±5%比 66±4%，p=0.014），右心室射血分数明显降低（25±2%比 51±3%，p<0.001），心肌周向应变降低（15.0±0.3%比 16.9±0.3%，p=0.007），右心室扩张（54±3 μL 比 40±3 μL，p=0.007）。Scgd(-/-)左心室的相邻区域之间的周向应变也表现出明显的时间不同步。我们的结果表明 Scgd(-/-) 小鼠在 8 个月时存在严重的心脏功能障碍。该研究确定了一组非侵入性标志物，可用于研究新型治疗剂在营养不良小鼠中的疗效。
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