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在体方法理解致心律失常性心肌病:动物模型的观点。

In Vivo Approaches to Understand Arrhythmogenic Cardiomyopathy: Perspectives on Animal Models.

机构信息

Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua, I-35128 Padua, Italy.

Department of Biology, University of Padua, I-35131 Padua, Italy.

出版信息

Cells. 2024 Jul 27;13(15):1264. doi: 10.3390/cells13151264.

Abstract

Arrhythmogenic cardiomyopathy (AC) is a hereditary cardiac disorder characterized by the gradual replacement of cardiomyocytes with fibrous and adipose tissue, leading to ventricular wall thinning, chamber dilation, arrhythmias, and sudden cardiac death. Despite advances in treatment, disease management remains challenging. Animal models, particularly mice and zebrafish, have become invaluable tools for understanding AC's pathophysiology and testing potential therapies. Mice models, although useful for scientific research, cannot fully replicate the complexity of the human AC. However, they have provided valuable insights into gene involvement, signalling pathways, and disease progression. Zebrafish offer a promising alternative to mammalian models, despite the phylogenetic distance, due to their economic and genetic advantages. By combining animal models with in vitro studies, researchers can comprehensively understand AC, paving the way for more effective treatments and interventions for patients and improving their quality of life and prognosis.

摘要

致心律失常性心肌病(AC)是一种遗传性心脏病,其特征是心肌细胞逐渐被纤维和脂肪组织取代,导致心室壁变薄、心室扩张、心律失常和心源性猝死。尽管治疗取得了进展,但疾病管理仍然具有挑战性。动物模型,特别是小鼠和斑马鱼,已成为了解 AC 病理生理学和测试潜在治疗方法的宝贵工具。小鼠模型虽然对科学研究很有用,但不能完全复制人类 AC 的复杂性。然而,它们为基因参与、信号通路和疾病进展提供了有价值的见解。尽管存在系统发育上的差异,但斑马鱼具有经济和遗传优势,是哺乳动物模型的一种很有前途的替代方法。通过将动物模型与体外研究相结合,研究人员可以全面了解 AC,为患者提供更有效的治疗和干预措施,提高他们的生活质量和预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8327/11311808/2ce37eb83735/cells-13-01264-g001.jpg

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