Signaling Pathways That Underlie Heart Disease

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Title Signaling Pathways That Underlie Heart Disease
Creator Yost, H.J.
Subject Diffusion of Innovation; Kabuki syndrome; Myocytes, Cardiac; Heart Defects, Congenital; Abnormalities, Multiple; Heart Failure; Neural Crest; Receptors, Notch; Receptor, Notch1; Signal Transduction; Serrate-Jagged Proteins; Knowledge Discovery
Keyword Health and Disease
Image Caption Notch signaling underlies the cardiovascular defects in the zebrafish model for Kabuki Syndrome.
Description Diseases affecting heart function exact an enormous toll on human health, but many of the genetic and molecular mechanisms underlying heart disease remain unknown. Yost and colleagues discovered novel roles for the same developmental signaling pathway in two seemingly unrelated sources of cardiac dysfunction: adult heart failure and embryonic heart malformation. In their first study, the team found that a unique population of heart muscle cells derived from embryonic neural crest are necessary for normal heart function. These cells produce a ligand for the Notch signaling receptor, Jag2b, and absence of either the cell population or the jag2b gene during development results in heart failure in adult fish. In their second study, they found that in a zebrafish model for Kabuki Syndrome, a congenital heart developmental disorder, Notch signaling is overactive. In a result with exciting implications for human patients, they showed that pharmacological inhibition of Notch signaling could restore normal heart development. Together, these two studies have identified a common mechanism that links diverse forms of heart disease.
Relation is Part of 2019
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date Digital 2020
Date 2019
Type Image
Format image/jpeg
Rights Management Copyright © 2021, University of Utah, All Rights Reserved
Language eng
ARK ark:/87278/s6h18r30
References 1.) Loss of embryonic neural crest derived cardiomyocytes causes adult onset hypertrophic cardiomyopathy in zebrafish. Abdul-Wajid S, Demarest BL, Yost HJ. Nat Commun. 2018 Nov;9(1):4603. 2.) Inhibition of Notch signaling rescues cardiovascular development in Kabuki Syndrome. Serrano MLA, Demarest BL, Tone-Pah-Hote T, Tristani-Firouzi M, Yost HJ. PLoS Biol. 2019 Sep;17(9):e3000087.
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ID 1589351
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