Lin Siyang, Zheng Yanping, Chen Meihuan, Xu Liangpu, Huang Hailong
Fujian Provincial Key Laboratory of Prenatal Diagnosis and Birth Defect, Medical Genetic Diagnosis and Therapy Center of Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
The School of Medical Technology and Engineering, Fujian Medical University, Fuzhou, China.
Front Physiol. 2024 Feb 26;15:1346173. doi: 10.3389/fphys.2024.1346173. eCollection 2024.
In Guangxi, Hainan, and Fujian Province in southern China, β-thalassemia is a frequent monogenic hereditary disorder that is primarily defined by hemolytic anemia brought on by inefficient erythropoiesis. It has been found that ineffective erythropoiesis in β-thalassemia is closely associated with a high accumulation of Reactive oxygen species, a product of oxidative stress, in erythroid cells. During recent years, ferroptosis is an iron-dependent lipid peroxidation that involves abnormalities in lipid and iron metabolism as well as reactive oxygen species homeostasis. It is a recently identified kind of programmed cell death. β-thalassemia patients experience increased iron release from reticuloendothelial cells and intestinal absorption of iron, ultimately resulting in iron overload. Additionally, the secretion of Hepcidin is inhibited in these patients. What counts is both ineffective erythropoiesis and ferroptosis in β-thalassemia are intricately linked to the iron metabolism and Reactive oxygen species homeostasis. Consequently, to shed further light on the pathophysiology of β-thalassemia and propose fresh ideas for its therapy, this paper reviews ferroptosis, ineffective erythropoiesis, and the way they interact.
在中国南方的广西、海南和福建省，β地中海贫血是一种常见的单基因遗传性疾病，主要表现为红细胞生成无效所导致的溶血性贫血。研究发现，β地中海贫血中红细胞生成无效与红系细胞中氧化应激产物活性氧的大量积累密切相关。近年来，铁死亡是一种铁依赖性脂质过氧化作用，涉及脂质和铁代谢异常以及活性氧稳态失衡。它是最近发现的一种程序性细胞死亡。β地中海贫血患者网状内皮细胞铁释放增加，肠道铁吸收增加，最终导致铁过载。此外，这些患者中hepcidin的分泌受到抑制。重要的是，β地中海贫血中的红细胞生成无效和铁死亡均与铁代谢和活性氧稳态密切相关。因此，为了进一步阐明β地中海贫血的病理生理学并为其治疗提出新思路，本文综述了铁死亡、红细胞生成无效及其相互作用方式。
