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Dysregulation of the EphrinB2−EphB4 ratio in pediatric cerebral arteriovenous malformations is associated with endothelial cell dysfunction in vitro and functions as a novel noninvasive biomarker in patients

Authors :
Katie Pricola Fehnel
David L. Penn
Micah Duggins-Warf
Maxwell Gruber
Steven Pineda
Julie Sesen
Alexander Moses-Gardner
Nishali Shah
Jessica Driscoll
David Zurakowski
Darren B. Orbach
Edward R. Smith
Source :
Experimental and Molecular Medicine, Vol 52, Iss 4, Pp 658-671 (2020)
Publication Year :
2020
Publisher :
Nature Publishing Group, 2020.

Abstract

Blood vessels: untangling the causes of brain vessel malformations Tangled blood vessel growths in the brain, known as arteriovenous malformations (AVMs), can be identified with a urine test, and the test protein may also help in treatment. AVMs often have no symptoms and can go undiagnosed, but when they rupture they can cause deadly brain hemorrhage. Better diagnostic tools and nonsurgical treatments are needed. Katie Fehnel and Edward Smith at Boston Children’s Hospital, USA, and co-workers identified an imbalance in a pair of signal/receptor proteins called ephrins in AVMs. Disturbing the balance of ephrin levels in blood vessel-forming cells disrupted growth, causing disorganized vessel formation with too many sprouts and insufficient junctions. Testing ephrin levels in patients’ urine reliably identified AVMs. These results offer a rapid and noninvasive new diagnostic tool and may help find new treatments for this mostly invisible and potentially fatal condition.

Subjects

Subjects :
Medicine
Biochemistry
QD415-436

Details

Language :
English
ISSN :
12263613 and 20926413
Volume :
52
Issue :
4
Database :
Directory of Open Access Journals
Journal :
Experimental and Molecular Medicine
Publication Type :
Academic Journal
Accession number :
edsdoj.fbd8e6855cdf49a49fe76bfabf3a0c48
Document Type :
article
Full Text :
https://doi.org/10.1038/s12276-020-0414-0