Article

Early atrial remodelling drives arrhythmia in Fabry Disease

2024-08-17

Abstract excerpt

<h4>ABSTRACT</h4> <h4>Background</h4> Fabry disease (FD) is an X-linked lysosomal storage disorder caused by α-galactosidase A (α-Gal A) deficiency, resulting in multi-organ accumulation of sphingolipid, namely globotriaosylceramide (Gb3). This triggers ventricular myocardial hypertrophy, fibrosis, and inflammation, driving arrhythmia and sudden death, a common cause of FD mortality. Atrial fibrillation (AF) is...

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Literature Corpus work: 68a1fa27-e298-516a-9e53-da37c18b8703
DOI: 10.1101/2024.08.13.607853

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