ArticleJournal of molecular and cellular cardiology2019
Phenotyping an adult zebrafish lamp2 cardiomyopathy model identifies mTOR inhibition as a candidate therapy.
Article in Journal of molecular and cellular cardiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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22 citing papers in PubMed, 36 citations in OpenAlex.
- Review
- Synapse specific alterations of autophagy are a hallmark of Danon disease.bioRxiv : the preprint server for biology · 2026Article
- Zebrafish in Cardiovascular Disease Research: from Model to Application.International journal of biological sciences · 2026Review
- Spns1-dependent endocardial lysosomal function drives valve morphogenesis through Notch1-signaling.iScience · 2024Article
- Review
- History and Perspective of LAMP-2 Deficiency (Danon Disease).Biomolecules · 2024Review
- Overview of oxidative stress and inflammation in diabetes.Journal of diabetes · 2024Review
- Allelic heterogeneity of TTNtv dilated cardiomyopathy can be modeled in adult zebrafish.JCI insight · 2024Article
- Research Progress on the Mechanism of Lysosome in Myocardial Ischemia-Reperfusion Injury Based on Autophagy.Reviews in cardiovascular medicine · 2024Review
- Danon Disease: EntireGenes · 2023Review
- Metabolic Maturation Exaggerates Abnormal Calcium Handling in aBiomolecules · 2022Article
- Evaluation of the transverse aortic constriction model in ICR and C57BL/6J mice.Frontiers in physiology · 2022Article
- Case Report: Danon Disease: Six Family Members and Literature Review.Frontiers in cardiovascular medicine · 2022Article
- Article
- Self-eating and Heart: The Emerging Roles of Autophagy in Calcific Aortic Valve Disease.Aging and disease · 2021Review
- TFEB Overexpression, Not mTOR Inhibition, Ameliorates RagCInternational journal of molecular sciences · 2021Article
- Sex-Specific Alterations in Cardiac DNA Methylation in Adult Mice by Perinatal Lead Exposure.International journal of environmental research and public health · 2021Article
- Zebrafish Heart Failure Models.Frontiers in cell and developmental biology · 2021Review
- Zebrafish as a model to study autophagy and its role in skeletal development and disease.Histochemistry and cell biology · 2020Review
- Gene therapy for cardiovascular diseases in China: basic research.Gene therapy · 2020Review
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Authors and funding
8 authors at 4 institutions in 2 countries.
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Abstract
Adult zebrafish is an emerging vertebrate model for studying genetic basis of cardiomyopathies; but whether the simple fish heart can model essential features of hypertrophic cardiomyopathy (HCM) remained unknown. Here, we report a comprehensive phenotyping of a lamp2 knockout (KO) mutant. LAMP2 encodes a lysosomal protein and is a causative gene of Danon disease that is characterized by HCM and massive autophagic vacuoles accumulation in the tissues. There is no effective therapy yet to treat this most lethal cardiomyopathy in the young. First, we did find the autophagic vacuoles accumulation in cardiac tissues from lamp2 KO. Next, through employing a set of emerging phenotyping tools, we revealed heart failure phenotypes in the lamp2 KO mutants, including decreased ventricular ejection fraction, reduced physical exercise capacity, blunted β-adrenergic contractile response, and enlarged atrium. We also noted changes of the following indices suggesting cardiac hypertrophic remodeling in lamp2 KO: a rounded heart shape, increased end-systolic ventricular volume and density of ventricular myocardium, elevated actomyosin activation kinetics together with increased maximal isometric tension at the level of cardiac myofibrils. Lastly, we assessed the function of lysosomal-localized mTOR on the lamp2-associated Danon disease. We found that haploinsufficiency of mtor was able to normalize some characteristics of the lamp2 KO, including ejection fraction, β-adrenergic response, and the actomyosin activation kinetics. In summary, we demonstrate the feasibility of modeling the inherited HCM in the adult zebrafish, which can be used to develop potential therapies.
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