Fabry Cardiomyopathy: Myocardial Fibrosis, Inflammation, and Down‐Regulation of Mannose‐6‐Phosphate Receptors Cause Low Accessibility to Enzyme Replacement Therapy

Fabry Cardiomyopathy: Myocardial Fibrosis, Inflammation, and Down‐Regulation of Mannose‐6‐Phosphate Receptors Cause Low Accessibility to Enzyme Replacement Therapy

Background The clinical impact of enzyme replacement therapy on advanced Fabry disease cardiomyopathy appears to be limited. The pathologic mechanisms involved are still unclear. Methods and Results Ten male patients with advanced Fabry disease cardiomyopathy on enzyme replacement therapy, whose dis...

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Bibliographic Details
Main Authors: Andrea Frustaci, Romina Verardo, Michele Magnocavallo, Emanuela Frustaci, Matteo Antonio Russo, Cristina Chimenti
Format: Article
Language:English
Published: Wiley 2025-02-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
enzyme replacement therapy
Fabry cardiomyopathy
Fabry disease
mannose‐6‐phosphate receptors
molecular rehabilitation
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.124.036815
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https://www.ahajournals.org/doi/10.1161/JAHA.124.036815

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