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Review

Dissecting the Role of the Extracellular Matrix in Heart Disease: Lessons from the Drosophila Genetic Model

Department of Biology, McMaster University, 1280 Main St. W., Hamilton, ON L8S 4K1, Canada
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Academic Editors: Sonja Fonfara and Lynne O’Sullivan
Vet. Sci. 2017, 4(2), 24; https://doi.org/10.3390/vetsci4020024
Received: 3 November 2016 / Revised: 15 February 2017 / Accepted: 20 April 2017 / Published: 24 April 2017
(This article belongs to the Special Issue Comparison of Cardiovascular Systems and Diseases Across Species)
The extracellular matrix (ECM) is a dynamic scaffold within organs and tissues that enables cell morphogenesis and provides structural support. Changes in the composition and organisation of the cardiac ECM are required for normal development. Congenital and age-related cardiac diseases can arise from mis-regulation of structural ECM proteins (Collagen, Laminin) or their receptors (Integrin). Key regulators of ECM turnover include matrix metalloproteinases (MMPs) and their inhibitors, tissue inhibitors of matrix metalloproteinases (TIMPs). MMP expression is increased in mice, pigs, and dogs with cardiomyopathy. The complexity and longevity of vertebrate animals makes a short-lived, genetically tractable model organism, such as Drosophila melanogaster, an attractive candidate for study. We survey ECM macromolecules and their role in heart development and growth, which are conserved between Drosophila and vertebrates, with focus upon the consequences of altered expression or distribution. The Drosophila heart resembles that of vertebrates during early development, and is amenable to in vivo analysis. Experimental manipulation of gene function in a tissue- or temporally-regulated manner can reveal the function of adhesion or ECM genes in the heart. Perturbation of the function of ECM proteins, or of the MMPs that facilitate ECM remodelling, induces cardiomyopathies in Drosophila, including cardiodilation, arrhythmia, and cardia bifida, that provide mechanistic insight into cardiac disease in mammals. View Full-Text
Keywords: cardiomyopathy; ECM; remodelling; Integrin; Collagen; MMP; TIMP; Drosophila; model organism; genetics cardiomyopathy; ECM; remodelling; Integrin; Collagen; MMP; TIMP; Drosophila; model organism; genetics
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MDPI and ACS Style

Hughes, C.J.R.; Jacobs, J.R. Dissecting the Role of the Extracellular Matrix in Heart Disease: Lessons from the Drosophila Genetic Model. Vet. Sci. 2017, 4, 24. https://doi.org/10.3390/vetsci4020024

AMA Style

Hughes CJR, Jacobs JR. Dissecting the Role of the Extracellular Matrix in Heart Disease: Lessons from the Drosophila Genetic Model. Veterinary Sciences. 2017; 4(2):24. https://doi.org/10.3390/vetsci4020024

Chicago/Turabian Style

Hughes, Chris J.R., and J. R. Jacobs 2017. "Dissecting the Role of the Extracellular Matrix in Heart Disease: Lessons from the Drosophila Genetic Model" Veterinary Sciences 4, no. 2: 24. https://doi.org/10.3390/vetsci4020024

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