Fibro-fatty remodelling in arrhythmogenic cardiomyopathy

Arwa Kohela; Eva van Rooij

doi:10.1007/s00395-022-00929-4

Fibro-fatty remodelling in arrhythmogenic cardiomyopathy

Hubrecht Institute

Onderzoeksoutput: Bijdrage aan wetenschappelijk tijdschrift/periodieke uitgave › Boek/film/artikelrecensie › Wetenschappelijk

7 Citaten (Scopus)

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Arrhythmogenic cardiomyopathy (AC) is an inherited disorder characterized by lethal arrhythmias and a risk to sudden cardiac death. A hallmark feature of AC is the progressive replacement of the ventricular myocardium with fibro-fatty tissue, which can act as an arrhythmogenic substrate further exacerbating cardiac dysfunction. Therefore, identifying the processes underlying this pathological remodelling would help understand AC pathogenesis and support the development of novel therapies. In this review, we summarize our knowledge on the different models designed to identify the cellular origin and molecular pathways underlying cardiac fibroblast and adipocyte cell differentiation in AC patients. We further outline future perspectives and how targeting the fibro-fatty remodelling process can contribute to novel AC therapeutics.

Originele taal-2	Engels
Pagina's (van-tot)	22
Tijdschrift	Basic Research in Cardiology
Volume	117
Nummer van het tijdschrift	1
DOI's	https://doi.org/10.1007/s00395-022-00929-4
Status	Gepubliceerd - 19 apr. 2022

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title = "Fibro-fatty remodelling in arrhythmogenic cardiomyopathy",

abstract = "Arrhythmogenic cardiomyopathy (AC) is an inherited disorder characterized by lethal arrhythmias and a risk to sudden cardiac death. A hallmark feature of AC is the progressive replacement of the ventricular myocardium with fibro-fatty tissue, which can act as an arrhythmogenic substrate further exacerbating cardiac dysfunction. Therefore, identifying the processes underlying this pathological remodelling would help understand AC pathogenesis and support the development of novel therapies. In this review, we summarize our knowledge on the different models designed to identify the cellular origin and molecular pathways underlying cardiac fibroblast and adipocyte cell differentiation in AC patients. We further outline future perspectives and how targeting the fibro-fatty remodelling process can contribute to novel AC therapeutics.",

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T1 - Fibro-fatty remodelling in arrhythmogenic cardiomyopathy

AU - Kohela, Arwa

AU - van Rooij, Eva

PY - 2022/4/19

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N2 - Arrhythmogenic cardiomyopathy (AC) is an inherited disorder characterized by lethal arrhythmias and a risk to sudden cardiac death. A hallmark feature of AC is the progressive replacement of the ventricular myocardium with fibro-fatty tissue, which can act as an arrhythmogenic substrate further exacerbating cardiac dysfunction. Therefore, identifying the processes underlying this pathological remodelling would help understand AC pathogenesis and support the development of novel therapies. In this review, we summarize our knowledge on the different models designed to identify the cellular origin and molecular pathways underlying cardiac fibroblast and adipocyte cell differentiation in AC patients. We further outline future perspectives and how targeting the fibro-fatty remodelling process can contribute to novel AC therapeutics.

AB - Arrhythmogenic cardiomyopathy (AC) is an inherited disorder characterized by lethal arrhythmias and a risk to sudden cardiac death. A hallmark feature of AC is the progressive replacement of the ventricular myocardium with fibro-fatty tissue, which can act as an arrhythmogenic substrate further exacerbating cardiac dysfunction. Therefore, identifying the processes underlying this pathological remodelling would help understand AC pathogenesis and support the development of novel therapies. In this review, we summarize our knowledge on the different models designed to identify the cellular origin and molecular pathways underlying cardiac fibroblast and adipocyte cell differentiation in AC patients. We further outline future perspectives and how targeting the fibro-fatty remodelling process can contribute to novel AC therapeutics.

KW - Arrhythmias, Cardiac/metabolism

KW - Cardiomyopathies/pathology

KW - Cell Differentiation

KW - Heart Ventricles/pathology

KW - Humans

KW - Myocardium/pathology

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M3 - Book/Film/Article review

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JO - Basic Research in Cardiology

JF - Basic Research in Cardiology

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Fibro-fatty remodelling in arrhythmogenic cardiomyopathy

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