{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T01:38:44Z","timestamp":1786498724886,"version":"build-2736575974"},"reference-count":209,"publisher":"Oxford University Press (OUP)","issue":"13","license":[{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004319","name":"Pfizer","doi-asserted-by":"publisher","award":["#67570063"],"award-info":[{"award-number":["#67570063"]}],"id":[{"id":"10.13039\/100004319","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004331","name":"Johnson & Johnson","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004331","id-type":"DOI","asserted-by":"publisher"}]},{"name":"European Union Horizon 2020","award":["874827"],"award-info":[{"award-number":["874827"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,10,28]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Amyloid light chain (AL) and transthyretin amyloidosis (ATTR)-induced cardiomyopathy are life-threatening protein misfolding disorders characterized by amyloid fibril deposition in the heart, which significantly impairs cardiac function. The lack of representative disease models has impeded progress in understanding the underlying mechanisms and hindered the discovery and development of specific biomarkers and effective therapies. To address this, researchers have developed various cell and animal models to recapitulate these diseases. In AL amyloidosis, cell and mouse models have highlighted the toxic effects of both soluble light chains (LCs) and LC-derived amyloid fibrils, such as lysosomal dysfunction, endoplasmic reticulum stress, and oxidative stress. Transgenic mouse models, particularly those without the mouse heavy chain and with amyloid seeds addition, have successfully replicated systemic AL amyloidosis, with clear effects on the heart. For ATTR amyloidosis, acid-induced transthyretin (TTR) fibrils induce cellular dysfunction, such as increased intracellular reactive oxygen species (ROS) level, disorganized sarcomere, and prolonged calcium handling in 2D cell models. Transgenic mouse models expressing human WT or variant TTR have offered insights into the development of amyloid cardiomyopathy, but challenges persist in fully replicating the human phenotype. This review offers a comprehensive overview of the significant advancements, challenges, and future perspectives in the development of various cell and animal models for studying AL and ATTR amyloidosis-induced cardiomyopathy, thereby providing valuable insights into disease pathophysiology, early accurate biomarkers identification, and development of novel therapies.<\/jats:p>","DOI":"10.1093\/cvr\/cvaf152","type":"journal-article","created":{"date-parts":[[2025,8,29]],"date-time":"2025-08-29T11:38:27Z","timestamp":1756467507000},"page":"1997-2013","source":"Crossref","is-referenced-by-count":4,"title":["<i>In vitro<\/i>\n                    and\n                    <i>in vivo<\/i>\n                    disease models of cardiac amyloidosis: progress, pitfalls, and potential"],"prefix":"10.1093","volume":"121","author":[{"given":"Jiabin","family":"Qin","sequence":"first","affiliation":[{"name":"Department of Cardiology, University Medical Center Utrecht , Heidelberglaan 100, 3508 GA, Utrecht, The Netherlands"},{"name":"Laboratory of Experimental Cardiology, Regenerative Medicine Center Utrecht, Circulatory Health Research Center, University Utrecht, University Medical Center Utrecht , Uppsalalaan 8, 3584 CT, Utrecht ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zeping","family":"Qiu","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine , 197 Ruijin 2nd Road, Shanghai 200025 ,","place":["People\u2019s Republic of China"]},{"name":"Institute of Cardiovascular Diseases, Shanghai Jiao Tong University School of Medicine , Shanghai ,","place":["People\u2019s Republic of China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yingze","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine , 197 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Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine , 197 Ruijin 2nd Road, Shanghai 200025 ,","place":["People\u2019s Republic of China"]},{"name":"Institute of Cardiovascular Diseases, Shanghai Jiao Tong University School of Medicine , Shanghai ,","place":["People\u2019s Republic of China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2713-686X","authenticated-orcid":false,"given":"Pim","family":"van der Harst","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Medical Center Utrecht , Heidelberglaan 100, 3508 GA, Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3139-8379","authenticated-orcid":false,"given":"Monique C","family":"Minnema","sequence":"additional","affiliation":[{"name":"Department of Hematology, University Medical Center Utrecht, University Utrecht , Utrecht ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joost P G","family":"Sluijter","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Medical Center Utrecht , Heidelberglaan 100, 3508 GA, Utrecht, The Netherlands"},{"name":"Laboratory of Experimental Cardiology, Regenerative Medicine Center Utrecht, Circulatory Health Research Center, University Utrecht, University Medical Center Utrecht , Uppsalalaan 8, 3584 CT, Utrecht ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9906-5047","authenticated-orcid":false,"given":"Alain","family":"van Mil","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Medical Center Utrecht , Heidelberglaan 100, 3508 GA, 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