Fibro-fatty remodelling in arrhythmogenic cardiomyopathy

Arwa Kohela; Eva van Rooij

doi:10.1007/s00395-022-00929-4

Fibro-fatty remodelling in arrhythmogenic cardiomyopathy

Arwa Kohela, Eva van Rooij

Hubrecht Institute

Research output: Contribution to journal/periodical › Book/Film/Article review › Scientific

9 Citations (Scopus)

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.

Original language	English
Pages (from-to)	22
Journal	Basic Research in Cardiology
Volume	117
Issue number	1
DOIs	https://doi.org/10.1007/s00395-022-00929-4
Publication status	Published - 19 Apr 2022

Keywords

Arrhythmias, Cardiac/metabolism
Cardiomyopathies/pathology
Cell Differentiation
Heart Ventricles/pathology
Humans
Myocardium/pathology

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10.1007/s00395-022-00929-4

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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

Y1 - 2022/4/19

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

U2 - 10.1007/s00395-022-00929-4

DO - 10.1007/s00395-022-00929-4

M3 - Book/Film/Article review

C2 - 35441328

SN - 0300-8428

VL - 117

SP - 22

JO - Basic Research in Cardiology

JF - Basic Research in Cardiology

IS - 1

ER -

Fibro-fatty remodelling in arrhythmogenic cardiomyopathy

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Keywords

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