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血浆中结合游离血红素的主要糖蛋白,可抵御氧化毒性,促进肝脏对铁的循环再利用。
蛋白生物学功能
血红素结合蛋白(Hemopexin)是一种血浆糖蛋白,对游离血红素具备已知最高的结合亲和力,在血管内溶血或细胞损伤过程中发挥关键清除蛋白的作用。该蛋白结构包含两个 β‑螺旋桨结构域,共同构成高亲和力血红素结合口袋;可中和血红素产生的氧化毒性,并通过 CD163 受体将血红素转运至肝脏,实现铁的循环再利用。作为急性期反应蛋白,炎症发生时血红素结合蛋白合成上调;但慢性溶血常会造成其血浆水平耗竭,进而加重组织损伤。
常见研究用途
血红素结合蛋白的缺乏或功能异常与镰状细胞病、β‑地中海贫血等溶血性贫血密切相关;游离血红素会诱导内皮激活、血管闭塞以及器官损伤。由于神经组织中血红素清除能力受损,该蛋白异常还与家族性癫痫的神经系统并发症相关;无法拮抗血红蛋白介导的炎症反应,也会增加脓毒症相关死亡风险。 在临床前模型中,补充血红素结合蛋白具备治疗潜力:可将镰状细胞小鼠的心血管功能损伤降低 50%,减轻溶血造成的肾脏损伤。该蛋白既可以作为溶血严重程度的生物标志物,也可作为潜在治疗试剂,用于减轻输血医学、动脉粥样硬化以及急性卟啉病中的血红素毒性。
常见研究方向:代谢研究、治疗药物研发、体外诊断(IVD)实验标准品、校准品与质控品、心血管研究、质谱分析、氧化应激研究、基于细胞的功能实验。
The primary plasma glycoprotein for binding free heme preventing oxidative toxicity and promoting iron recycling by the liver.
Role of protein in biology
Hemopexin is a plasma glycoprotein with the highest known binding affinity for free heme, functioning as a critical scavenger during intravascular hemolysis or cellular injury. Structurally, it comprises two β-propeller domains that form a high-affinity heme-binding pocket, enabling it to neutralize heme's oxidative toxicity and facilitate its transport to the liver for iron recycling via CD163 receptors. As an acute-phase reactant, hemopexin synthesis increases during inflammation, though chronic hemolysis often depletes its levels, exacerbating tissue damage.
Common research uses
Deficiencies or dysfunction in hemopexin are implicated in hemolytic anemias such as sickle cell disease and β-thalassemia, where unbound heme drives endothelial activation, vaso-occlusion, and organ damage. It also correlates with neurologic complications in familial epilepsy due to impaired heme clearance in neural tissues and contributes to sepsis-related mortality by failing to counteract hemoglobin-mediated inflammation. Therapeutically, hemopexin supplementation shows promise in preclinical models, reducing cardiovascular dysfunction in sickle cell mice by 50% and mitigating renal injury in hemolysis. It serves as both a biomarker for hemolytic severity and a potential therapeutic agent to attenuate heme toxicity in transfusion medicine, atherosclerosis, and acute porphyrias.
Common uses include metabolic studies, therapeutics research, IVD Assay standards, calibrators and controls, cardiovascular research, mass spectrometry, oxidative stress research, cell based assays.
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