Article
Myofilament degradation and dysfunction of human cardiomyocytes in Fabry disease.
The American journal of pathology - 1 Jun 2008
Chimenti Cristina, Hamdani Nazha, Boontje Nicky M, DeCobelli Francesco, Esposito Antonio, Bronzwaer Jean G F, Stienen Ger J M, Russo Matteo A, Paulus Walter J, Frustaci Andrea, van der Velden Jolanda
Abstract excerpt
Early detection of myocardial dysfunction in Fabry disease (FD) cardiomyopathy suggests the contribution of myofilament structural alterations. Six males with untreated FD cardiomyopathy submitted to cardiac studies, including tissue Doppler imaging and left ventricular endomyocardial biopsy. Active and resting tensions before and after treatment with protein kinase A (PKA) were determined in isolated...
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