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Cardiac energy metabolism is disturbed in Fabry disease and improves with enzyme replacement therapy using recombinant human galactosidase A.

Abstract:

AIMS: In vitro studies have shown impairment of energy metabolism in cardiac fibroblasts from Fabry patients. A recent in vivo study reported an association between cardiac energy metabolism and increased myocardial mass in Fabry patients. We therefore assessed possible disturbances of cardiac energy metabolism in Fabry patients by in vivo (31)P-MR-spectroscopy. Additionally, the effect of enzyme replacement therapy (ERT) on cardiac energetics was tested. METHODS AND RESULTS: Twenty-three pa...

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Publication status:
Published

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Publisher copy:
10.1093/eurjhf/hfq211

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Journal:
European journal of heart failure
Volume:
13
Issue:
3
Pages:
278-283
Publication date:
2011-03-05
DOI:
EISSN:
1879-0844
ISSN:
1388-9842
URN:
uuid:ab8be4d7-91b5-45b0-95ae-bb5db369b1b0
Source identifiers:
124879
Local pid:
pubs:124879

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