关键词: DMT1 ZIP14 ZIP8 ferroportin iron transport mitoferrin pathogenic mutations transferrin

Mesh : Iron Biological Transport Membrane Proteins Cell Membrane Lysosomes

来  源:   DOI:10.3390/biom13081172   PDF(Pubmed)

Abstract:
Iron is an essential transition metal for its involvement in several crucial biological functions, the most notable being oxygen storage and transport. Due to its high reactivity and potential toxicity, intracellular and extracellular iron levels must be tightly regulated. This is achieved through transport systems that mediate cellular uptake and efflux both at the level of the plasma membrane and on the membranes of lysosomes, endosomes and mitochondria. Among these transport systems, the key players are ferroportin, the only known transporter mediating iron efflux from cells; DMT1, ZIP8 and ZIP14, which on the contrary, mediate iron influx into the cytoplasm, acting on the plasma membrane and on the membranes of lysosomes and endosomes; and mitoferrin, involved in iron transport into the mitochondria for heme synthesis and Fe-S cluster assembly. The focus of this review is to provide an updated view of the physiological role of these membrane proteins and of the pathologies that arise from defects of these transport systems.
摘要:
铁是一种重要的过渡金属,因为它参与了几个关键的生物学功能,最值得注意的是氧气储存和运输。由于其高反应性和潜在毒性,细胞内和细胞外铁水平必须严格调节。这是通过在质膜水平和溶酶体膜上介导细胞摄取和流出的转运系统实现的。内体和线粒体。在这些运输系统中,关键参与者是ferroportin,唯一已知的介导铁流出细胞的转运蛋白;DMT1,ZIP8和ZIP14,相反,介导铁流入细胞质,作用于质膜以及溶酶体和核内体的膜;和有丝分裂铁蛋白,参与铁转运到线粒体中进行血红素合成和Fe-S簇组装。这篇综述的重点是提供这些膜蛋白的生理作用以及这些运输系统缺陷引起的病理的最新观点。
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