دورية أكاديمية
Myocardial Fibrosis in Hypertrophic Cardiomyopathy: A Perspective from Fibroblasts
العنوان: | Myocardial Fibrosis in Hypertrophic Cardiomyopathy: A Perspective from Fibroblasts |
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المؤلفون: | Maja Schlittler, Peter P. Pramstaller, Alessandra Rossini, Marzia De Bortoli |
المصدر: | International Journal of Molecular Sciences, Vol 24, Iss 19, p 14845 (2023) |
بيانات النشر: | MDPI AG, 2023. |
سنة النشر: | 2023 |
المجموعة: | LCC:Biology (General) LCC:Chemistry |
مصطلحات موضوعية: | hypertrophic cardiomyopathy, myocardial fibrosis, cardiac fibroblast, myofibroblasts, TGF-β, Biology (General), QH301-705.5, Chemistry, QD1-999 |
الوصف: | Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease and the leading cause of sudden cardiac death in young people. Mutations in genes that encode structural proteins of the cardiac sarcomere are the more frequent genetic cause of HCM. The disease is characterized by cardiomyocyte hypertrophy and myocardial fibrosis, which is defined as the excessive deposition of extracellular matrix proteins, mainly collagen I and III, in the myocardium. The development of fibrotic tissue in the heart adversely affects cardiac function. In this review, we discuss the latest evidence on how cardiac fibrosis is promoted, the role of cardiac fibroblasts, their interaction with cardiomyocytes, and their activation via the TGF-β pathway, the primary intracellular signalling pathway regulating extracellular matrix turnover. Finally, we summarize new findings on profibrotic genes as well as genetic and non-genetic factors involved in the pathophysiology of HCM. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 1422-0067 1661-6596 |
Relation: | https://www.mdpi.com/1422-0067/24/19/14845; https://doaj.org/toc/1661-6596; https://doaj.org/toc/1422-0067 |
DOI: | 10.3390/ijms241914845 |
URL الوصول: | https://doaj.org/article/f38225b90f914baaab36357ed4fe0b58 |
رقم الأكسشن: | edsdoj.f38225b90f914baaab36357ed4fe0b58 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 14220067 16616596 |
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DOI: | 10.3390/ijms241914845 |