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Oxidative Stress Contributes to Endothelial Dysfunction in Mouse Models of Hereditary Hemorrhagic Telangiectasia
Joint Authors
Jerkic, Mirjana
Sotov, Valentin
Letarte, Michelle
Source
Oxidative Medicine and Cellular Longevity
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-12-25
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Natural & Life Sciences (Multidisciplinary)
Biology
Abstract EN
Hereditary hemorrhagic telangiectasia (HHT) is a vascular dysplasia caused by mutations in endoglin (ENG; HHT1) or activin receptor-like kinase (ALK1; HHT2) genes, coding for transforming growth factor-β (TGF-β) superfamily receptors.
We demonstrated previously that endoglin and ALK1 interact with endothelial NO synthase (eNOS) and affect its activation.
Endothelial cells deficient in endoglin or ALK1 proteins show eNOS uncoupling, reduced NO, and increased reactive oxygen species (ROS) production.
In this study, we measured NO and H2O2 levels in several organs of adult Eng and Alk1 heterozygous mice, to ascertain whether decreased NO and increased ROS production is a generalized manifestation of HHT.
A significant reduction in NO and increase in ROS production were found in several organs, known to be affected in patients.
ROS overproduction in mutant mice was attributed to eNOS, as it was L-NAME inhibitable.
Mitochondrial ROS contribution, blocked by antimycin, was highest in liver while NADPH oxidase, inhibited by apocynin, was a major source of ROS in the other tissues.
However, there was no difference in antimycin- and apocynin-inhibitable ROS production between mutant and control mice.
Our results indicate that eNOS-derived ROS contributes to endothelial dysfunction and likely predisposes to disease manifestations in several organs of HHT patients.
American Psychological Association (APA)
Jerkic, Mirjana& Sotov, Valentin& Letarte, Michelle. 2012. Oxidative Stress Contributes to Endothelial Dysfunction in Mouse Models of Hereditary Hemorrhagic Telangiectasia. Oxidative Medicine and Cellular Longevity،Vol. 2012, no. 2012, pp.1-9.
https://search.emarefa.net/detail/BIM-490592
Modern Language Association (MLA)
Jerkic, Mirjana…[et al.]. Oxidative Stress Contributes to Endothelial Dysfunction in Mouse Models of Hereditary Hemorrhagic Telangiectasia. Oxidative Medicine and Cellular Longevity No. 2012 (2012), pp.1-9.
https://search.emarefa.net/detail/BIM-490592
American Medical Association (AMA)
Jerkic, Mirjana& Sotov, Valentin& Letarte, Michelle. Oxidative Stress Contributes to Endothelial Dysfunction in Mouse Models of Hereditary Hemorrhagic Telangiectasia. Oxidative Medicine and Cellular Longevity. 2012. Vol. 2012, no. 2012, pp.1-9.
https://search.emarefa.net/detail/BIM-490592
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-490592