Review
Cell & Tissue Engineering
Jiangtao Li, Xin Feng, Xiang Wei
Summary: This review summarizes the methods of establishing cardiomyocyte models using induced pluripotent stem cells (iPSCs) and the progress in genome editing techniques. Because the currently cultured iPSC-CMs are immature, researchers have attempted various methods to promote their maturation. Many researchers have established iPSC-CM models of hypertrophic cardiomyopathy (HCM) and used diverse methods for measurements.
STEM CELL RESEARCH & THERAPY
(2022)
Review
Cell Biology
Xue Jiang, Yihuan Chen, Xiaofeng Liu, Lingqun Ye, Miao Yu, Zhenya Shen, Wei Lei, Shijun Hu
Summary: Researchers have discovered the contribution of genetic defects to the pathogenesis of primary cardiomyopathy and tried to explain the pathogenesis of these diseases by establishing various disease models. The advent of human induced pluripotent stem cells (hiPSCs) provides an unprecedented opportunity to further investigate the pathogenic mechanisms of inherited cardiomyopathies in vitro using patient-specific hiPSC-derived cardiomyocytes. The development of defects in genetically modified animal models differs notably from human diseases at the molecular level.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
Li Feng, Jianhua Zhang, ChangHwan Lee, Gina Kim, Fang Liu, Andrew J. Petersen, Evi Lim, Corey L. Anderson, Kate M. Orland, Gail A. Robertson, Lee L. Eckhardt, Craig T. January, Timothy J. Kamp
Summary: This study investigated the pathogenesis of the hERG1-H70R variant in long QT syndrome type 2 using patient-specific iPSC-CMs, revealing a mechanism involving impaired trafficking of hERG1a and maintained expression of hERG1b leading to subunit imbalance and reduced I-Kr.
CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Elisa Giacomelli, Luca Sala, Dorien Ward-van Oostwaard, Milena Bellin
Summary: Wild-type and LQT1 hiPSCs were differentiated into various cell types to create 3D cardiac microtissues, successfully recapitulating the typical features of LQTS. The use of a 96-well multi-electrode array proved effective in detecting electrical changes in the 3D tissues.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
(2021)
Review
Cardiac & Cardiovascular Systems
Chrishan J. A. Ramachandra, Jasper Chua, Shuo Cong, Myu Mai Ja Kp, Winston Shim, Joseph C. Wu, Derek J. Hausenloy
Summary: Normal cardiac functions depend on continuous energy supply. Metabolic disturbances and impaired mitochondrial bioenergetics underlie various cardiac diseases, but specific treatments are lacking. Patient-derived iPSCs show promise for studying cardiomyopathies and novel therapies.
CARDIOVASCULAR RESEARCH
(2021)
Review
Cell Biology
Mario G. Pavez-Giani, Lukas Cyganek
Summary: This review summarizes recent advances in iPSC-based disease modeling of mitochondrial cardiomyopathies and explores the patho-mechanistic insights as well as new therapeutic approaches. Researchers have used iPSC technology to recapitulate major characteristics of mitochondrial cardiomyopathies, providing a powerful platform for drug development and testing of new therapies.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Annika Winbo, Suganeya Ramanan, Emily Eugster, Annika Rydberg, Stefan Jovinge, Jonathan R. Skinner, Johanna M. Montgomery
Summary: Sympathetic activation is known to trigger life-threatening cardiac events in long QT syndrome type 1 (LQT1). This study investigated human induced pluripotent stem cell (hiPSC)-derived sympathetic neurons (SNs) from LQT1 patients, revealing increased neurotransmission and excitability. The findings suggest that the arrhythmogenic potential in LQT1 is not limited to cardiomyocytes, highlighting the importance of sympathetic neuronal hyperactivity in the pathogenesis of sympathetically triggered arrhythmias.
AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
Assad Shiti, Gil Arbil, Naim Shaheen, Irit Huber, Noga Setter, Lior Gepstein
Summary: The study aims to generate a unique model for studying atrial arrhythmias related to short QT syndrome (SQTS) using patient-specific human induced pluripotent stem cells (hiPSCs), atrial-specific differentiation schemes, two-dimensional tissue modeling, optical mapping, and drug testing.
JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY
(2023)
Review
Cardiac & Cardiovascular Systems
Dihui Cai, Zequn Zheng, Xiaojun Jin, Yin Fu, Lichao Cen, Jiachun Ye, Yongfei Song, Jiangfang Lian
Summary: This article introduces the second most common subtype of long QT syndrome, LQT2, and its pathogenic gene mutations, as well as the advantages and applications of using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) as a model for studying LQT2.
JOURNAL OF CARDIOVASCULAR TRANSLATIONAL RESEARCH
(2023)
Article
Cell & Tissue Engineering
Ning Ge, Min Liu, Rui Li, Nicholas M. Allen, Joseph Galvin, Sanbing Shen, Timothy O'Brien, Terence W. Prendiville
Summary: This study presents an optimized and rapid protocol for introducing a heterozygous LQT3-specific variant into healthy control hiPSCs. The researchers successfully screened hiPSCs carrying the LQT3 pathogenic variant and confirmed its normal genetic background and functionality. Additionally, protocols for differentiating hiPSCs into cardiomyocytes and evaluating their electrophysiological characteristics are provided.
STEM CELL REVIEWS AND REPORTS
(2023)
Review
Biology
Takeshi Tokuyama, Razan Elfadil Ahmed, Nawin Chanthra, Tatsuya Anzai, Hideki Uosaki
Summary: Mitochondrial cardiomyopathy (MCM) is characterized by oxidative phosphorylation disorder in the heart, with over 100 genetic variants associated with the disease. Patient-specific induced pluripotent stem cell (iPSC)-derived cardiomyocytes (iPSC-CMs) are an attractive experimental platform for modeling cardiovascular diseases and predicting drug efficacy for such diseases. This study introduces the pathological and therapeutic studies of MCM using iPSC-CMs, discussing current questions and research strategies in the field.
Article
Cell & Tissue Engineering
Gulen Guney-Esken, Ozgur Dogus Erol, Burcu Pervin, Gulben Gurhan Sevinc, Tamer Onder, Elif Bilgic, Petek Korkusuz, Aysen Gunel-Ozcan, Duygu Uckan-Cetinkaya, Fatima Aerts-Kaya
Summary: GS-2 is a rare autosomal recessive immune deficiency syndrome caused by a mutation in the RAB27A gene. This study successfully generated iPSC from 3 GS-2 patients using lentiviral vectors, which expressed pluripotency markers and differentiated into cells from all three germ layers. The iPSCs can be used for future studies on novel treatments and the pathophysiology of GS-2 disease.
STEM CELL RESEARCH & THERAPY
(2021)
Article
Cell Biology
Ying Zhang, Jun Wei, Jiani Cao, Kehua Zhang, Yaojin Peng, Hongkui Deng, Jiuhong Kang, Guangjin Pan, Yong Zhang, Boqiang Fu, Shijun Hu, Jie Na, Yan Liu, Lei Wang, Lingmin Liang, Huanxin Zhu, Yu Zhang, Zi-Bing Jin, Jie Hao, Aijin Ma, Tongbiao Zhao, Junying Yu
Summary: "Requirements for Human-Induced Pluripotent Stem Cells" is the first set of guidelines in China on human-induced pluripotent stem cells. It provides detailed technical requirements and aims to promote international standardization.
CELL PROLIFERATION
(2022)
Article
Cell & Tissue Engineering
Tongbin Ding, Wenli Zhu, Xiaowei Li, Liguo Jian
Summary: Long QT syndrome is a common hereditary arrhythmia, and mutations in AKAP9 gene can cause LQT11. In this study, a patient-derived induced pluripotent stem cell line was established as a cell disease model for understanding the pathogenesis of LQT11.
STEM CELL RESEARCH
(2022)
Article
Cell & Tissue Engineering
Chi Zhang, Jian Shi, Zhen Zhang, Qianqian Zhao, Manting Xie, Ying Chen, Qiuling Xiang
Summary: In this study, peripheral blood mononuclear cells from a patient with hypertrophic cardiomyopathy were reprogrammed into pluripotent stem cells using Sendai-virus. The established cell line showed strong expression of pluripotency markers and differentiation potential, providing a valuable tool for investigating the pathogenesis and therapeutic strategies of HCM.
STEM CELL RESEARCH
(2022)
Editorial Material
Cell & Tissue Engineering
Javier Linares, Estibaliz Arellano-Viera, Olalla Iglesias-Garcia, Carmen Ferreira, Elena Iglesias, Gloria Abizanda, Felipe Prosper, Xonia Carvajal-Vergara
STEM CELL RESEARCH
(2016)
Article
Cell & Tissue Engineering
Alvaro Munoz-Lopez, Damia Romero-Moya, Cristina Prieto, Veronica Ramos-Mejia, Antonio Agraz-Doblas, Ignacio Varela, Marcus Buschbeck, Anna Palau, Xonia Carvajal-Vergara, Alessandra Giorgetti, Anthony Ford, Majlinda Lako, Isabel Granada, Neus Ruiz-Xiville, Sandra Rodriguez-Perales, Raul Torres-Ruiz, Ronald W. Stam, Jose Luis Fuster, Mario F. Fraga, Mahito Nakanishi, Gianni Cazzaniga, Michela Bardini, Isabel Cobo, Gustavo F. Bayon, Agustin F. Fernandez, Clara Bueno, Pablo Menendez
Article
Cell & Tissue Engineering
Giulia Coppiello, Gloria Abizanda, Natalia Aguado, Elena Iglesias, Estibaliz Arellano-Viera, Juan R. Rodriguez-Madoz, Xonia Carvajal-Vergara, Felipe Prosper, Xabier L. Aranguren
STEM CELL RESEARCH
(2017)
Article
Cardiac & Cardiovascular Systems
Bo Lin, Jong Kim, Yanxin Li, Haiying Pan, Xonia Carvajal-Vergara, Guy Salama, Tao Cheng, Yong Li, Cecilia W. Lo, Lei Yang
CARDIOVASCULAR RESEARCH
(2012)
Article
Cell & Tissue Engineering
Dung-Fang Lee, Jie Su, Yen-Sin Ang, Xonia Carvajal-Vergara, Sonia Mulero-Navarro, Carlos F. Pereira, Julian Gingold, Hung-Liang Wang, Ruiying Zhao, Ana Sevilla, Henia Darr, Andrew J. K. Williamson, Betty Chang, Xiaohong Niu, Francesca Aguilo, Elsa R. Flores, Yuh-Pyng Sher, Mien-Chie Hung, Anthony D. Whetton, Bruce D. Gelb, Kateri A. Moore, Hans-Willem Snoeck, Avi Ma'ayan, Christoph Schaniel, Ihor R. Lemischka
Article
Multidisciplinary Sciences
Xonia Carvajal-Vergara, Ana Sevilla, Sunita L. D'Souza, Yen-Sin Ang, Christoph Schaniel, Dung-Fang Lee, Lei Yang, Aaron D. Kaplan, Eric D. Adler, Roye Rozov, YongChao Ge, Ninette Cohen, Lisa J. Edelmann, Betty Chang, Avinash Waghray, Jie Su, Sherly Pardo, Klaske D. Lichtenbelt, Marco Tartaglia, Bruce D. Gelb, Ihor R. Lemischka
Article
Cell & Tissue Engineering
Julio Castano, Pablo Menendez, Cristina Bruzos-Cidon, Marco Straccia, Amaia Sousa, Lorea Zabaleta, Nerea Vazquez, Amaia Zubiarrain, Kai-Christian Sonntag, Luisa Ugedo, Xonia Carvajal-Vergara, Josep Maria Canals, Maria Torrecilla, Rosario Sanchez-Pernaute, Alessandra Giorgetti
Article
Cell & Tissue Engineering
Javier Linares, Leyre Lopez-Muneta, Estibaliz Arellano-Viera, Purificacion Ripalda-Cemborain, Elena Iglesias, Gloria Abizanda, Xabier L. Aranguren, Felipe Prosper, Xonia Carvajal-Vergara
STEM CELL RESEARCH
(2018)
Article
Cell & Tissue Engineering
Estibaliz Arellano-Viera, Lorea Zabaleta, Julio Castano, Garikoitz Azkona, Xonia Carvajal-Vergara, Alessandra Giorgetti
STEM CELL RESEARCH
(2019)
Article
Agriculture, Dairy & Animal Science
Marta Moya-Jodar, Giulia Coppiello, Juan Roberto Rodriguez-Madoz, Gloria Abizanda, Paula Barlabe, Amaia Vilas-Zornoza, Asier Ullate-Agote, Chiara Luongo, Ernesto Rodriguez-Tobon, Sergio Navarro-Serna, Evelyne Paris-Oller, Maria Oficialdegui, Xonia Carvajal-Vergara, Laura Ordovas, Felipe Prosper, Francisco Alberto Garcia-Vazquez, Xabier L. Aranguren
Summary: The generation of organs using pluripotent stem cells combined with genetically engineered pig embryos through blastocyst complementation could provide a solution to the worldwide shortage of transplantable organs. In this study, a protocol to create vascular-disabled pig embryos using the CRISPR/Cas9 system was optimized, which could contribute to the generation of rejection-free humanized organs in pigs.
Article
Biochemistry & Molecular Biology
Gloria Abizanda, Leyre Lopez-Muneta, Javier Linares, Luis Ramos, Arantxa Baraibar-Churio, Miriam Bobadilla, Elena Iglesias, Giulia Coppiello, Purificacion Ripalda-Cemborain, Xabier L. Aranguren, Felipe Prosper, Ana Perez-Ruiz, Xonia Carvajal-Vergara
Summary: The study demonstrates for the first time that preconditioning the infarcted cardiac tissue with local irradiation can substantially enhance cell engraftment.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Leyre Lopez-Muneta, Josu Miranda-Arrubla, Xonia Carvajal-Vergara
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2020)
Meeting Abstract
Hematology
P. Maiso, E. M. Ocio, X. Carvajal-Vergara, M. Garayoa, J. Carlos Montero, P. Atadja, A. Pandiella, J. E. San Miguel
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
(2007)
Article
Cell Biology
Maria Gomez-Benito, Patricia Balsas, Xonia Carvajal-Vergara, Atanasio Pandiella, Alberto Anel, Isabel Marzo, Javier Naval
CELLULAR SIGNALLING
(2007)