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| Verfasst von: | Fan, Xuehui [VerfasserIn]  |
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| | Yang, Guoqiang [VerfasserIn] |
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| | Duru, Firat [VerfasserIn] |
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| | Grilli, Maurizio [VerfasserIn] |
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| | Akın, Ibrahim [VerfasserIn] |
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| | Zhou, Xiao-Bo [VerfasserIn] |
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| | Saguner, Ardan Muammer [VerfasserIn] |
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| | El-Battrawy, Ibrahim [VerfasserIn] |
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| Titel: | Arrhythmogenic cardiomyopathy |
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| Titelzusatz: | from preclinical models to genotype-phenotype correlation and pathophysiology |
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| Verf.angabe: | Xuehui Fan, Guoqiang Yang, Firat Duru, Maurizio Grilli, Ibrahim Akin, Xiaobo Zhou, Ardan Muammer Saguner, Ibrahim EI-Battrawy |
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| E-Jahr: | 2023 |
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| Jahr: | November 2023 |
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| Umfang: | 26 S. |
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| Illustrationen: | Illustrationen |
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| Fussnoten: | Online veröffentlicht: 20. September 2023 ; Gesehen am 12.03.2024 |
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| Titel Quelle: | Enthalten in: Stem cell reviews and reports |
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| Ort Quelle: | New York, NY : Springer, 2009 |
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| Jahr Quelle: | 2023 |
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| Band/Heft Quelle: | 19(2023), 8 vom: Nov., Seite 2683-2708 |
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| ISSN Quelle: | 2629-3277 |
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| Abstract: | Arrhythmogenic cardiomyopathy (ACM) is a hereditary myocardial disease characterized by the replacement of the ventricular myocardium with fibrous fatty deposits. ACM is usually inherited in an autosomal dominant pattern with variable penetrance and expressivity, which is mainly related to ventricular tachyarrhythmia and sudden cardiac death (SCD). Importantly, significant progress has been made in determining the genetic background of ACM due to the development of new techniques for genetic analysis. The exact molecular pathomechanism of ACM, however, is not completely clear and the genotype-phenotype correlations have not been fully elucidated, which are useful to predict the prognosis and treatment of ACM patients. Different gene-targeted and transgenic animal models, human-induced pluripotent stem cell-derived cardiomyocyte (hiPSC-CM) models, and heterologous expression systems have been developed. Here, this review aims to summarize preclinical ACM models and platforms promoting our understanding of the pathogenesis of ACM and assess their value in elucidating the ACM genotype-phenotype relationship. |
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| DOI: | doi:10.1007/s12015-023-10615-0 |
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| URL: | Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.
kostenfrei: Volltext: https://doi.org/10.1007/s12015-023-10615-0 |
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| | kostenfrei: Volltext: https://link.springer.com/article/10.1007/s12015-023-10615-0 |
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| | DOI: https://doi.org/10.1007/s12015-023-10615-0 |
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| Datenträger: | Online-Ressource |
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| Sprache: | eng |
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| Sach-SW: | Animal models |
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| | Arrhythmogenic cardiomyopathy |
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| | Desmosomes |
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| | Human-induced pluripotent stem cell-derived cardiomyocytes |
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| | Pathophysiology |
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| K10plus-PPN: | 1883191912 |
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| Verknüpfungen: | → Zeitschrift |
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Arrhythmogenic cardiomyopathy / Fan, Xuehui [VerfasserIn]; November 2023 (Online-Ressource)
