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World J Stem Cells. Mar 26, 2023; 15(3): 71-82
Published online Mar 26, 2023. doi: 10.4252/wjsc.v15.i3.71
Disease modeling of desmosome-related cardiomyopathy using induced pluripotent stem cell-derived cardiomyocytes
Shuichiro Higo
Shuichiro Higo, Department of Medical Therapeutics for Heart Failure, Osaka University Graduate School of Medicine, Suita 565-0871, Japan
Author contributions: Higo S wrote the manuscript; the author has read and approved the final manuscript.
Supported by JSPS KAKENHI, No. 20K21602, No. 21H02915, and No. 22K19526; the Japan Agency for Medical Research and Development, No. 21bm0804008h0005; the Cell Science Research Foundation; the Grant for Basic Research of the Japanese Circulation Society (2018); and SENSHIN Medical Research Foundation.
Conflict-of-interest statement: Department of Medical Therapeutics for Heart Failure is a Joint Research Department with TOA EIYO Pharmaceutical Company. Dr. Higo reports grants from TOA EIYO Pharmaceutical Company, during the conduct of the study.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Shuichiro Higo, MD, PhD, Specially Appointed Associate Professor, Department of Medical Therapeutics for Heart Failure, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita 565-0871, Japan. higo-s@cardiology.med.osaka-u.ac.jp
Received: December 16, 2022
Peer-review started: December 16, 2022
First decision: February 3, 2023
Revised: February 10, 2023
Accepted: March 15, 2023
Article in press: March 15, 2023
Published online: March 26, 2023
Core Tip

Core Tip: Prevention of advanced heart failure caused by cardiomyopathy is an urgent unmet need in the field of cardiovascular medicine. Desmosome, a cell-to-cell junctional component, maintains the structural integrity of heart tissues. Genetic mutations in desmosomal genes cause desmosome-related cardiomyopathy, an intractable disease refractory to standard medical therapies. This review introduces the recent advances in disease modeling of desmosome-related cardiomyopathy caused by PKP2 mutations using induced pluripotent stem cell-derived cardiomyocytes.