肌营养不良蛋白
纤维化
杜氏肌营养不良
mdx鼠标
心肌病
医学
肌营养不良
心脏纤维化
病理
心功能曲线
内分泌学
内科学
渗透(HVAC)
心力衰竭
热力学
物理
作者
Christel van Erp,D. S. Loch,Nicola Laws,Andrea L. Trebbin,Andrew Hoey
摘要
Abstract The dystrophin‐deficient (mdx) mouse remains the most commonly used model for Duchenne muscular dystrophy (DMD). Mdx mice show a predominantly covert cardiomyopathy, the hallmark of which is fibrosis. We compared mdx and normal mice at six ages (3, 6, 9, 12, 15, and 18 months) using in vivo assessment of cardiac function, selective collagen staining, and measures of TGF‐β mRNA, Evans blue dye infiltration, macrophage infiltration, and aortic wall thickness. Clear temporal progression was demonstrated, including early fragility of cardiomyocyte membranes, which has an unrelated impact on cardiac function but is associated with macrophage infiltration and fibrosis. Aortic wall thickness is less in older mdx mice. Mdx mice display impaired responses to inotropic challenge from a young age; this is indicative of altered adrenoreceptor function. We draw attention to the paradox of ongoing fibrosis in mdx hearts without a strong molecular signature (in the form of TGF‐β mRNA expression). Muscle Nerve, 2010
科研通智能强力驱动
Strongly Powered by AbleSci AI