Critical Role of Hemopexin Mediated Cytoprotection in the Pathophysiology of Sickle Cell Disease.
Critical Role of Hemopexin Mediated Cytoprotection in the Pathophysiology of Sickle Cell Disease.
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DOI：
10.3390/ijms22126408
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发表时间：
2021-06-15
影响因子：
5.6
通讯作者：
Doré S
中科院分区：
文献类型：
--
作者：
Ashouri R;Fangman M;Burris A;Ezenwa MO;Wilkie DJ;Doré S
循环血红素结合蛋白是负责血红素清除的主要蛋白质;因此，它是对抗由游离血红素的存在诱导的有害炎症和氧化应激的全身性战斗剂。溶血结合蛋白的这种作用在溶血病理生理学中至关重要。在这篇综述中，我们概述了目前的研究，关于如何在镰状细胞病，其特点是病理性聚集的红细胞和过度溶血的血红素结合蛋白的动态活性有牵连。这种病理生理学导致诸如急性肾损伤、血管闭塞、缺血性中风、疼痛危象和由于游离血红素和血红蛋白的存在而加重的肺动脉高压的症状。本文综述了镰状细胞病小鼠、大鼠和豚鼠模型的体内研究，以及人体样本的研究。总之，目前的研究表明，血红素结合蛋白可能对这些症状具有保护作用，并且在镰状细胞病患者中纠正耗尽的血红素结合蛋白可以改善或预防症状。本综述中的数据表明，应进行进一步的临床前和临床研究，以揭示血液结合蛋白在病理状态下的通路，以评估其作为镰状细胞病和相关血红蛋白病的生物标志物和治疗的潜在临床功能。
Circulating hemopexin is the primary protein responsible for the clearance of heme; therefore, it is a systemic combatant against deleterious inflammation and oxidative stress induced by the presence of free heme. This role of hemopexin is critical in hemolytic pathophysiology. In this review, we outline the current research regarding how the dynamic activity of hemopexin is implicated in sickle cell disease, which is characterized by a pathological aggregation of red blood cells and excessive hemolysis. This pathophysiology leads to symptoms such as acute kidney injury, vaso-occlusion, ischemic stroke, pain crises, and pulmonary hypertension exacerbated by the presence of free heme and hemoglobin. This review includes in vivo studies in mouse, rat, and guinea pig models of sickle cell disease, as well as studies in human samples. In summary, the current research indicates that hemopexin is likely protective against these symptoms and that rectifying depleted hemopexin in patients with sickle cell disease could improve or prevent the symptoms. The data compiled in this review suggest that further preclinical and clinical research should be conducted to uncover pathways of hemopexin in pathological states to evaluate its potential clinical function as both a biomarker and therapy for sickle cell disease and related hemoglobinopathies.
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