Serum Biomarkers of Endothelial Dysfunction in Fabry Associated Cardiomyopathy

Frontiers in Cardiovascular Medicine
Jefferson LosoMonica Patten

Abstract

Background: Fabry disease (FD) is characterized by early development of vasculopathy and endothelial dysfunction. However, it is unclear whether these findings also play a pivotal role in cardiac manifestation. As Fabry cardiomyopathy (FC) is the leading cause of death in FD, we aimed to gather a better insight in pathological mechanisms of the disease. Methods: Serum samples were obtained from 17 healthy controls, 15 FD patients with and 7 without FC. FC was defined by LV wall thickening of >12 mm in cardiac magnetic resonance imaging and serum level of proBNP, high sensitive Troponin T (hsT), and globotriaosylsphingosine (lyso-GB3) were obtained. A multiplex ELISA-Assay for 23 different angiogenesis markers was performed in pooled samples. Markers showing significant differences among groups were further analyzed in single samples using specific Elisa antibody assays. L-homoarginine (hArg), L-arginine, asymmetric (ADMA), and symmetric Dimethylarginine (SDMA) were quantified by liquid chromatography-mass spectrometry. Results: Angiostatin and matrix metalloproteinase 9 (MMP-9) were elevated in FD patients compared to controls independently of the presence of FC (angiostatin: 98 ± 25 vs. 75 ± 15 ng/mL; p = 0.001; MMP-9: 8.0 ± 3...Continue Reading

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Citations

Jul 14, 2020·Current Pathobiology Reports·Roberto I MotaEdward M Bahnson
Feb 13, 2021·International Journal of Molecular Sciences·Daniela Sorriento, Guido Iaccarino
Dec 15, 2020·Trends in Neurosciences·Sophie QuickAnna Williams
May 1, 2021·Journal of Clinical Medicine·Clara Carnicer-CáceresGuillem Pintos-Morell
Jun 8, 2021·Advances in Pharmacological and Pharmaceutical Sciences·Cosimo A StamerraAmirhossein Sahebkar

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Methods Mentioned

BETA
Enzyme-Linked
Antibody
ELISAs
ELISA

Software Mentioned

cvi42®
Image [UNK]

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