Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Taghli-Lamallem, Ouarda, Plantié, Emilie, Jagla, Krzysztof
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5715700/
https://www.ncbi.nlm.nih.gov/pubmed/29367558
http://dx.doi.org/10.3390/jcdd3010007
_version_ 1783283816587067392
author Taghli-Lamallem, Ouarda
Plantié, Emilie
Jagla, Krzysztof
author_facet Taghli-Lamallem, Ouarda
Plantié, Emilie
Jagla, Krzysztof
author_sort Taghli-Lamallem, Ouarda
collection PubMed
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.
format Online
Article
Text
id pubmed-5715700
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-57157002018-01-19 Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly Taghli-Lamallem, Ouarda Plantié, Emilie Jagla, Krzysztof J Cardiovasc Dev Dis Review 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. MDPI 2016-02-18 /pmc/articles/PMC5715700/ /pubmed/29367558 http://dx.doi.org/10.3390/jcdd3010007 Text en © 2016 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons by Attribution (CC-BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Taghli-Lamallem, Ouarda
Plantié, Emilie
Jagla, Krzysztof
Drosophila in the Heart of Understanding Cardiac Diseases: Modeling Channelopathies and Cardiomyopathies in the Fruitfly
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 Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5715700/
https://www.ncbi.nlm.nih.gov/pubmed/29367558
http://dx.doi.org/10.3390/jcdd3010007
work_keys_str_mv AT taghlilamallemouarda drosophilaintheheartofunderstandingcardiacdiseasesmodelingchannelopathiesandcardiomyopathiesinthefruitfly
AT plantieemilie drosophilaintheheartofunderstandingcardiacdiseasesmodelingchannelopathiesandcardiomyopathiesinthefruitfly
AT jaglakrzysztof drosophilaintheheartofunderstandingcardiacdiseasesmodelingchannelopathiesandcardiomyopathiesinthefruitfly