N Lund, H Wieboldt, L Fischer, N Muschol, F Braun, T Huber, D Sorriento, G Iaccarino, K Müllerleile, E Tahir, G Adam, P Kirchhof, L Fabritz, M Patten
INTRODUCTION: Fabry's disease is an X-linked lysosomal storage disorder caused by reduced activity of α-galactosidase A (GAL), leading to premature death on account of renal, cardiac, and vascular organ failure. Accumulation of the GAL substrate globotriaosylceramide (Gb3) in endothelial and smooth muscle cells is associated with early vascular cell damage, suggesting endothelial dysfunction as a driver of cardiorenal organ failure. Here, we studied the vascular expression of the key angiogenic factors, VEGFα and its antagonist angiostatin, in Fabry α-GAL-Tg/KO mice and determined circulating VEGFα and angiostatin serum levels in patients with Fabry's disease and healthy controls...
2024: Frontiers in Cardiovascular Medicine