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Models of dilated cardiomyopathy in the mouse and the hamster
1Department of Medicine, University of California San Diego, La Jolla 92093-0613B, USA.
Insights
Small animal models, particularly mice, are crucial for understanding dilated cardiomyopathy (DCM), a heart muscle disorder. These models help investigate genetic roles and mimic human DCM phenotypes for therapeutic insights.
Area of Science:
- Cardiology
- Genetics
- Molecular Biology
Background:
- Dilated cardiomyopathy (DCM) is a significant heart muscle disorder.
- It involves atrial and ventricular dilation, dysfunction, and heart failure.
- Animal models are vital for studying DCM pathogenesis.
Purpose of the Study:
- To review small animal models of dilated cardiomyopathy (DCM).
- To categorize these models based on genetic mutations and affected pathways.
- To highlight their utility in understanding human DCM.
Main Methods:
- Focus on genetically engineered and naturally occurring small animal models, especially mice.
- Categorization based on intrasarcomeric/extrasarcomeric cytoskeletal abnormalities.
- Examination of models involving intracellular signaling pathways (e.g., beta-adrenergic, calcium regulation).
Main Results:
- Animal models successfully mimic human DCM phenotypes.
- Specific gene mutations and pathway disruptions are linked to DCM.
- Modifications in signaling pathways can influence DCM development or severity.
Conclusions:
- Small animal models, particularly mice, are indispensable for DCM research.
- These models elucidate the role of genetic factors and signaling pathways in DCM.
- Insights from animal models inform our understanding of myocyte adaptation and survival mechanisms.
Abstract:
Dilated cardiomyopathy (DCM) is a heart muscle disorder characterized by atrial and ventricular dilation often with relative wall thinning, severe systolic and diastolic ventricular dysfunction, and frequent findings of heart failure. Using genetically engineered mice, a number of studies have attempted to determine the role of specific genes, as well as to mimic the phenotype of human DCM. Naturally occurring and acquired animal models of DCM also have been investigated. In this brief review, we will focus on small animal models of DCM, particularly those in the mouse, together with some comments on the autosomal-recessive cardiomyopathy of the hamster. These animal models can be categorized into several general groups in accordance with the presumed role of the gene mutation involved, including intrasarcomeric and extrasarcomeric cytoskeletal abnormalities, which resemble some forms of hereditary human DCM, and overexpression or disruption of genes that control molecules participating in intracellular signaling pathways, including the beta-adrenergic system and calcium regulation. Modifications in the latter two pathways can cause or alleviate DCM in animal models, suggesting their importance in myocyte adaptive and survival mechanisms.