Kumfu Sirinart, Fucharoen Suthat, Chattipakorn Siriporn C, Chattipakorn Nipon
1 Faculty of Medicine, Cardiac Electrophysiology Research and Training Center, Chiang Mai University, Chiang Mai 50200, Thailand.
2 Faculty of Medicine, Department of Physiology, Cardiac Electrophysiology Unit, Chiang Mai University, Chiang Mai 50200, Thailand.
Exp Biol Med (Maywood). 2017 Jun;242(11):1126-1135. doi: 10.1177/1535370217708977. Epub 2017 May 9.
Beta-thalassemia is an inherited hemoglobin disorder caused by reduced or absent synthesis of the beta globin chains of hemoglobin. This results in variable outcomes ranging from clinically asymptomatic to severe anemia, which then typically requires regular blood transfusion. These regular blood transfusions can result in an iron overload condition. The iron overload condition can lead to iron accumulation in various organs, especially in the heart, leading to iron overload cardiomyopathy, which is the major cause of mortality in patients with thalassemia. In the past decades, there is no doubt that the use of β-thalassemic mice as a study model to investigate the pathophysiology of iron overload cardiomyopathy and the role of various pharmacological interventions, has shed some light in understanding this serious complication and in improving the associated cardiac dysfunction. In this review, the effects that iron overload has on the hearts of β-thalassemic mice under conditions of iron overload as well as the efficacy of pharmacological interventions to combat these adverse effects on the heart are reviewed and discussed. The in-depth understanding of biomolecular alterations in the heart of these iron overload thalassemic mice will help give guidance for more effective therapeutic approaches in the near future. Impact statement Iron overload cardiomyopathy is a major cause of morbidity and mortality in patients with thalassemia. Since investigation of iron overload cardiomyopathy in thalassemia patients has many limitations, a search for an animal model for this condition has been ongoing for decades. In the past decades, there is no doubt that the use of β-thalassemic mice as a study model to investigate the pathophysiology of iron overload cardiomyopathy and the role of various pharmacological interventions, has shed some light in understanding this serious complication and in improving the associated cardiac dysfunction. In this review, the effects of iron overload on the hearts of β-thalassemic mice under conditions of iron overload as well as the efficacy of pharmacological interventions to combat these adverse effects on the heart are reviewed and discussed.
β地中海贫血是一种遗传性血红蛋白疾病，由血红蛋白β珠蛋白链合成减少或缺失引起。这会导致从临床无症状到严重贫血等不同的结果，严重贫血通常需要定期输血。这些定期输血会导致铁过载状况。铁过载状况会导致铁在各个器官中积累，尤其是在心脏，从而导致铁过载性心肌病，这是地中海贫血患者死亡的主要原因。在过去几十年中，毫无疑问，使用β地中海贫血小鼠作为研究模型来研究铁过载性心肌病的病理生理学以及各种药物干预的作用，为理解这一严重并发症和改善相关心脏功能障碍提供了一些线索。在这篇综述中，我们回顾并讨论了铁过载在铁过载条件下对β地中海贫血小鼠心脏的影响以及对抗这些心脏不良反应的药物干预的疗效。对这些铁过载地中海贫血小鼠心脏中生物分子变化的深入了解将有助于在不久的将来为更有效的治疗方法提供指导。影响声明铁过载性心肌病是地中海贫血患者发病和死亡的主要原因。由于对地中海贫血患者铁过载性心肌病的研究存在许多局限性，数十年来一直在寻找这种疾病的动物模型。在过去几十年中，毫无疑问，使用β地中海贫血小鼠作为研究模型来研究铁过载性心肌病的病理生理学以及各种药物干预的作用，为理解这一严重并发症和改善相关心脏功能障碍提供了一些线索。在这篇综述中，我们回顾并讨论了铁过载在铁过载条件下对β地中海贫血小鼠心脏的影响以及对抗这些心脏不良反应的药物干预的疗效。
