کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
10833601 1065799 2014 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Improvement of cardiac function by short-term enzyme replacement therapy in a murine model of cardiomyopathy associated with Hunter syndrome evaluated by serial echocardiography with speckle tracking 2-D strain analysis
ترجمه فارسی عنوان
بهبود عملکرد قلبی توسط درمان جایگزین آنزیمی کوتاه مدت در یک مدل موش از کریو مپیوپاتی همراه با سندرم هانتر با استفاده از اکوکاردیوگرافی سریالی با تجزیه و تحلیل اسپین دو جهش
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
چکیده انگلیسی
Cardiac systolic function is significantly decreased in a proportion of patients with Hunter syndrome. This study was performed to evaluate the change in myocardial function associated with enzyme replacement therapy (ERT) in a mouse model of cardiomyopathy associated with Hunter syndrome. Thirty 9-week-old iduronate-2-sulfatase (IDS) knockout mice received either intravenous injection of human recombinant IDS (ERT group, N = 15) or saline (control group, N = 15) for 5 weeks. Echocardiography was performed at baseline and after treatment. Echocardiographic parameters of left ventricular (LV) systolic function and 2-dimensional radial and circumferential strain were assessed. At follow-up, there was a significant increase in LV fractional shortening and radial and circumferential strain in the ERT group only. Notable myocardial fibrosis was observed in the control group only. In the murine model of Hunter syndrome, ERT exerts beneficial effects on cardiac function, which can be evaluated by serial echocardiographic evaluation including 2-dimensional strain analysis.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular Genetics and Metabolism - Volume 112, Issue 3, July 2014, Pages 218-223
نویسندگان
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