کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
8619088 1567597 2018 36 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
The Genetic and Molecular Bases for Hypertrophic Cardiomyopathy: The Role for Calcium Sensitization
ترجمه فارسی عنوان
مبانی ژنتیک و مولکولی برای کاردیومیوپاتی هیپرتروفیک: نقش حساسیت به کلسیم
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی بیهوشی و پزشکی درد
چکیده انگلیسی
Hypertrophic cardiomyopathy (HCM) affects millions of people around the world as one of the most common genetic heart disorders and leads to cardiac ischemia, heart failure, dysfunction of other organ systems, and increased risk for sudden unexpected cardiac deaths. HCM can be caused by single-point mutations, insertion or deletion mutations, or truncation of cardiac myofilament proteins. The molecular mechanism that leads to disease progression and presentation is still poorly understood, despite decades of investigations. However, recent research has made dramatic advances in the understanding of HCM disease development. Studies have shown that increased calcium sensitivity is a universal feature in HCM. At the molecular level, increased crossbridge force (or power) generation resulting in hypercontractility is the prominent feature. Thus, calcium sensitization/hypercontractility is emerging as the primary stimulus for HCM disease development and phenotypic expression. Cross-bridge inhibition has been shown to halt HCM presentation, and myofilament desensitization appears to reduce lethal arrhythmias in animal models of HCM. These advances in basic research will continue to deepen the knowledge of HCM pathogenesis and are beginning to revolutionize the management of HCM.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Cardiothoracic and Vascular Anesthesia - Volume 32, Issue 1, February 2018, Pages 478-487
نویسندگان
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