Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cells
Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy characterized by the replacement of myocardium by fibro-fatty infiltration and cardiomyocyte loss. ACM predisposes to a high risk for ventricular arrhythmias. ACM has initially been defined as a desmosomal disease because most of the...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2023-05-01
|
| Series: | Frontiers in Physiology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fphys.2023.1191965/full |
| _version_ | 1797829251763798016 |
|---|---|
| author | J. B. Reisqs A. Moreau Y. Sleiman M. Boutjdir M. Boutjdir M. Boutjdir S. Richard P. Chevalier P. Chevalier |
| author_facet | J. B. Reisqs A. Moreau Y. Sleiman M. Boutjdir M. Boutjdir M. Boutjdir S. Richard P. Chevalier P. Chevalier |
| author_sort | J. B. Reisqs |
| collection | DOAJ |
| description | Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy characterized by the replacement of myocardium by fibro-fatty infiltration and cardiomyocyte loss. ACM predisposes to a high risk for ventricular arrhythmias. ACM has initially been defined as a desmosomal disease because most of the known variants causing the disease concern genes encoding desmosomal proteins. Studying this pathology is complex, in particular because human samples are rare and, when available, reflect the most advanced stages of the disease. Usual cellular and animal models cannot reproduce all the hallmarks of human pathology. In the last decade, human-induced pluripotent stem cells (hiPSC) have been proposed as an innovative human cellular model. The differentiation of hiPSCs into cardiomyocytes (hiPSC-CM) is now well-controlled and widely used in many laboratories. This hiPSC-CM model recapitulates critical features of the pathology and enables a cardiomyocyte-centered comprehensive approach to the disease and the screening of anti-arrhythmic drugs (AAD) prescribed sometimes empirically to the patient. In this regard, this model provides unique opportunities to explore and develop new therapeutic approaches. The use of hiPSC-CMs will undoubtedly help the development of precision medicine to better cure patients suffering from ACM. This review aims to summarize the recent advances allowing the use of hiPSCs in the ACM context. |
| first_indexed | 2024-04-09T13:18:31Z |
| format | Article |
| id | doaj.art-13d4b7ec665b414f96b8d7fac466257e |
| institution | Directory Open Access Journal |
| issn | 1664-042X |
| language | English |
| last_indexed | 2024-04-09T13:18:31Z |
| publishDate | 2023-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Physiology |
| spelling | doaj.art-13d4b7ec665b414f96b8d7fac466257e2023-05-11T11:21:08ZengFrontiers Media S.A.Frontiers in Physiology1664-042X2023-05-011410.3389/fphys.2023.11919651191965Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cellsJ. B. Reisqs0A. Moreau1Y. Sleiman2M. Boutjdir3M. Boutjdir4M. Boutjdir5S. Richard6P. Chevalier7P. Chevalier8Cardiovascular Research Program, VA New York Harbor Healthcare System, Brooklyn, NY, United StatesUniversité de Montpellier, Institut National de la Santé et de la Recherche Médicale, Centre National de la Recherche Scientifique, PhyMedExp, Montpellier, FranceCardiovascular Research Program, VA New York Harbor Healthcare System, Brooklyn, NY, United StatesCardiovascular Research Program, VA New York Harbor Healthcare System, Brooklyn, NY, United StatesDepartment of Medicine, Cell Biology and Pharmacology, State University of New York Downstate Health Sciences University, NY, United StatesDepartment of Medicine, New York University School of Medicine, NY, United StatesUniversité de Montpellier, Institut National de la Santé et de la Recherche Médicale, Centre National de la Recherche Scientifique, PhyMedExp, Montpellier, FranceNeuromyogene Institute, Claude Bernard University, Lyon 1, Villeurbanne, FranceService de Rythmologie, Hospices Civils de Lyon, Lyon, FranceArrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy characterized by the replacement of myocardium by fibro-fatty infiltration and cardiomyocyte loss. ACM predisposes to a high risk for ventricular arrhythmias. ACM has initially been defined as a desmosomal disease because most of the known variants causing the disease concern genes encoding desmosomal proteins. Studying this pathology is complex, in particular because human samples are rare and, when available, reflect the most advanced stages of the disease. Usual cellular and animal models cannot reproduce all the hallmarks of human pathology. In the last decade, human-induced pluripotent stem cells (hiPSC) have been proposed as an innovative human cellular model. The differentiation of hiPSCs into cardiomyocytes (hiPSC-CM) is now well-controlled and widely used in many laboratories. This hiPSC-CM model recapitulates critical features of the pathology and enables a cardiomyocyte-centered comprehensive approach to the disease and the screening of anti-arrhythmic drugs (AAD) prescribed sometimes empirically to the patient. In this regard, this model provides unique opportunities to explore and develop new therapeutic approaches. The use of hiPSC-CMs will undoubtedly help the development of precision medicine to better cure patients suffering from ACM. This review aims to summarize the recent advances allowing the use of hiPSCs in the ACM context.https://www.frontiersin.org/articles/10.3389/fphys.2023.1191965/fullhiPSC-CMarrhythmogenic cardiomyopathytransdifferentiationpersonalized medicineelectrophysiology |
| spellingShingle | J. B. Reisqs A. Moreau Y. Sleiman M. Boutjdir M. Boutjdir M. Boutjdir S. Richard P. Chevalier P. Chevalier Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cells Frontiers in Physiology hiPSC-CM arrhythmogenic cardiomyopathy transdifferentiation personalized medicine electrophysiology |
| title | Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cells |
| title_full | Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cells |
| title_fullStr | Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cells |
| title_full_unstemmed | Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cells |
| title_short | Arrhythmogenic cardiomyopathy as a myogenic disease: highlights from cardiomyocytes derived from human induced pluripotent stem cells |
| title_sort | arrhythmogenic cardiomyopathy as a myogenic disease highlights from cardiomyocytes derived from human induced pluripotent stem cells |
| topic | hiPSC-CM arrhythmogenic cardiomyopathy transdifferentiation personalized medicine electrophysiology |
| url | https://www.frontiersin.org/articles/10.3389/fphys.2023.1191965/full |
| work_keys_str_mv | AT jbreisqs arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT amoreau arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT ysleiman arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT mboutjdir arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT mboutjdir arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT mboutjdir arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT srichard arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT pchevalier arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells AT pchevalier arrhythmogeniccardiomyopathyasamyogenicdiseasehighlightsfromcardiomyocytesderivedfromhumaninducedpluripotentstemcells |