Key result
Peg1 gene knockout in mice alters myocardial trabeculation and density, resembling human ventricular noncompaction.
Why the study?
The function and expression pattern of the imprinted gene Peg1 (Mest) in the developing heart and its role in myocardial trabeculation were unknown.
The Peg1 (Mest) gene plays a role in the spatial and temporal pattern of myocardial trabeculation during mouse heart development, and its absence mimics features of ventricular noncompaction.
Peg1 loss models noncompaction in mice; hypothesis-generating for its role in human disease and requires validation.
Peg1 (Mest) is an imprinted gene of unknown function widely expressed in the mouse embryo, predominantly in cells of the mesodermal lineage. We have revealed a restricted expression pattern within the developing heart. Initial uniform expression throughout the linear heart tube subsequently becomes restricted, primarily to the developing myocardial trabeculae of both the atria and ventricles, where it persists into late development. Expression in the atrial appendage myocardium precedes the emergence of trabeculae (pectinate muscles), and occurs earlier and to a greater extent on the right than on the left, reflecting the spatial and temporal pattern of trabeculation. Analysis of myocardial morphology in mice lacking the Peg1 gene, which are viable and appear grossly normal, reveals a subtle alteration in the pattern of trabeculation: an increase in thickness and reduction in density of the compact myocardium, similar to that seen in the human cardiomyopathy ventricular noncompaction.
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King et al. (2002) studied Myocardial trabeculation in developing mouse heart. Peg1 (Mest) gene knockout vs. Wild-type mice (implied) was evaluated on Myocardial morphology and pattern of trabeculation. Mice lacking the Peg1 gene exhibited altered trabeculation with increased thickness and reduced density of the compact myocardium, resembling human ventricular noncompaction.
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