Zandman-Goddard Gisele, Shoenfeld Yehuda
Department of Medicine C, Wolfson Medical Center, Israel.
Autoimmun Rev. 2007 Aug;6(7):457-63. doi: 10.1016/j.autrev.2007.01.016. Epub 2007 Feb 16.
Iron, an essential element for many important cellular functions in all living organisms, can catalyze the formation of potentially toxic free radicals. Excessive iron is sequestered by ferritin in a nontoxic and readily available form in a cell. Ferritin is composed of 24 subunits of different proportions of two functionally distinct subunits: ferritin H and L. The expression of ferritin is under delicate control and is regulated at both the transcriptional and post-transcriptional levels by iron, cytokines, hormones, and oxidative stress. Mutations in the ferritin gene cause the hereditary hyperferritinemia-cataract syndrome and neuroferritinopathy. Hyperferritinemia is associated with inflammation, infections, and malignancies. While elevated levels of ferritin are characteristic of adult-onset Still's disease and hemophagocytic syndrome, both associated with inflammation, it has scantly been evaluated in other autoimmune diseases. In this review, we describe ferritin structure and function, hyperferritinemia in disease states and in autoimmune diseases.
铁是所有生物中许多重要细胞功能所必需的元素,它能催化潜在有毒自由基的形成。过量的铁以无毒且易于利用的形式被细胞中的铁蛋白螯合。铁蛋白由24个亚基组成,这24个亚基由两种功能不同的亚基(铁蛋白H和L)按不同比例构成。铁蛋白的表达受到精细调控,在转录和转录后水平上都受到铁、细胞因子、激素和氧化应激的调节。铁蛋白基因突变会导致遗传性高铁蛋白血症 - 白内障综合征和神经铁蛋白病。高铁蛋白血症与炎症、感染和恶性肿瘤有关。虽然铁蛋白水平升高是成人斯蒂尔病和噬血细胞综合征的特征,这两种疾病都与炎症有关,但在其他自身免疫性疾病中对其研究甚少。在本综述中,我们描述了铁蛋白的结构和功能、疾病状态及自身免疫性疾病中的高铁蛋白血症。