Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly

Cardiovascular diseases and, among them, channelopathies and cardiomyopathies are a major cause of death worldwide. The molecular and genetic defects underlying these cardiac disorders are complex, leading to a large range of structural and functional heart phenotypes. Identification of molecular an...

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Main Authors: Ouarda Taghli-Lamallem, Emilie Plantié, Krzysztof Jagla
Format: Article
Language:English
Published: MDPI AG 2016-02-01
Series:Journal of Cardiovascular Development and Disease
Subjects:
Online Access:http://www.mdpi.com/2308-3425/3/1/7
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author Ouarda Taghli-Lamallem
Emilie Plantié
Krzysztof Jagla
author_facet Ouarda Taghli-Lamallem
Emilie Plantié
Krzysztof Jagla
author_sort Ouarda Taghli-Lamallem
collection DOAJ
description Cardiovascular diseases and, among them, channelopathies and cardiomyopathies are a major cause of death worldwide. The molecular and genetic defects underlying these cardiac disorders are complex, leading to a large range of structural and functional heart phenotypes. Identification of molecular and functional mechanisms disrupted by mutations causing channelopathies and cardiomyopathies is essential to understanding the link between an altered gene and clinical phenotype. The development of animal models has been proven to be efficient for functional studies in channelopathies and cardiomyopathies. In particular, the Drosophila model has been largely applied for deciphering the molecular and cellular pathways affected in these inherited cardiac disorders and for identifying their genetic modifiers. Here we review the utility and the main contributions of the fruitfly models for the better understanding of channelopathies and cardiomyopathies. We also discuss the investigated pathological mechanisms and the discoveries of evolutionarily conserved pathways which reinforce the value of Drosophila in modeling human cardiac diseases.
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spelling doaj.art-b1e6a82d682b47b4a2a389605644d8832022-12-22T03:35:10ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252016-02-0131710.3390/jcdd3010007jcdd3010007Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the FruitflyOuarda Taghli-Lamallem0Emilie Plantié1Krzysztof Jagla2GReD (Genetics, Reproduction and Development laboratory), INSERM U1103, CNRS UMR6293, University of Clermont-Ferrand, 28 place Henri-Dunant, 63000 Clermont-Ferrand, FranceGReD (Genetics, Reproduction and Development laboratory), INSERM U1103, CNRS UMR6293, University of Clermont-Ferrand, 28 place Henri-Dunant, 63000 Clermont-Ferrand, FranceGReD (Genetics, Reproduction and Development laboratory), INSERM U1103, CNRS UMR6293, University of Clermont-Ferrand, 28 place Henri-Dunant, 63000 Clermont-Ferrand, FranceCardiovascular diseases and, among them, channelopathies and cardiomyopathies are a major cause of death worldwide. The molecular and genetic defects underlying these cardiac disorders are complex, leading to a large range of structural and functional heart phenotypes. Identification of molecular and functional mechanisms disrupted by mutations causing channelopathies and cardiomyopathies is essential to understanding the link between an altered gene and clinical phenotype. The development of animal models has been proven to be efficient for functional studies in channelopathies and cardiomyopathies. In particular, the Drosophila model has been largely applied for deciphering the molecular and cellular pathways affected in these inherited cardiac disorders and for identifying their genetic modifiers. Here we review the utility and the main contributions of the fruitfly models for the better understanding of channelopathies and cardiomyopathies. We also discuss the investigated pathological mechanisms and the discoveries of evolutionarily conserved pathways which reinforce the value of Drosophila in modeling human cardiac diseases.http://www.mdpi.com/2308-3425/3/1/7human cardiac diseaseDrosophilachannelopathiescardiomyopathiessarcomeric proteinscytoskeletal proteinsK+ channels
spellingShingle Ouarda Taghli-Lamallem
Emilie Plantié
Krzysztof Jagla
Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly
Journal of Cardiovascular Development and Disease
human cardiac disease
Drosophila
channelopathies
cardiomyopathies
sarcomeric proteins
cytoskeletal proteins
K+ channels
title Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly
title_full Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly
title_fullStr Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly
title_full_unstemmed Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly
title_short Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly
title_sort drosophila in the heart of understanding cardiac diseases modeling channelopathies and cardiomyopathies in the fruitfly
topic human cardiac disease
Drosophila
channelopathies
cardiomyopathies
sarcomeric proteins
cytoskeletal proteins
K+ channels
url http://www.mdpi.com/2308-3425/3/1/7
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AT krzysztofjagla drosophilaintheheartofunderstandingcardiacdiseasesmodelingchannelopathiesandcardiomyopathiesinthefruitfly