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First published online August 16, 2005

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Big-hearted mice provide clues to cardiac failure

Familial hypertrophic cardiomyopathy (FHC), an autosomal dominant disorder, is caused by mutations in cardiac muscle filament proteins. One FHC variant, which is characterized by mid-left ventricular obstruction due to papillary muscle hypertrophy, is caused by an E22K mutation in the ventricular myosin regulatory light chain (RLC). To investigate the functional consequences of this mutation, Danuta Szczesna-Cordary and co-workers have generated transgenic mice overexpressing the E22K mutant of human ventricular RLC (see p. 3675). Histologically, the hearts of these animals resemble those of human patients although, surprisingly, the mice have apparently normal echocardiograms. Physiological studies reveal an increased calcium sensitivity of ATPase activity and force development in the cardiac muscles from transgenic E22K-RLC mice when compared with muscles from mice carrying wild-type human RLC. These and results from other model systems indicate that the E22K mutation causes structural perturbations in RLC that affect the myocardium's calcium binding properties and trigger abnormal heart function. This, the authors suggest, may initiate a hypertrophic process and lead to heart failure.

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Related articles in JCS:

The E22K mutation of myosin RLC that causes familial hypertrophic cardiomyopathy increases calcium sensitivity of force and ATPase in transgenic mice

Danuta Szczesna-Cordary, Georgianna Guzman, Jiaju Zhao, Olga Hernandez, Jianqin Wei, and Zoraida Diaz-Perez
JCS 2005 118: 3675-3683. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in JCS Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Social Bookmarking                         What's this?