Le Gall Jean-Yves, Jouanolle Anne-Marie, Mosser Jean, David Véronique
l'Académie nationale de médecine, Laboratoire de Génétique Moléculaire CHR, UMR 6061 CNRS Faculté de Médecine, Rennes.
Bull Acad Natl Med. 2005 Nov;189(8):1635-47; discussion 1647.
Iron, associated with proteins and enzymes, mainly as heminic groups and Fe/S clusters, is essential for oxygen transport and many other biological functions. Systemic iron homeostasis is essentially a closed system. There is no regulated mechanism of iron excretion, for example through the liver or kidneys: iron losses occur only through bleeding and by shedding of mucosal and skin cells. These losses are compensated for by intestinal absorption. In contrast, iron absorption is tightly regulated. Three recently identified proteins, named HFE, hepcidin and hemojuvelin, play an important role in this regulation. About 20 other proteins have been shown to play a part in iron metabolism, often through studies of genetic diseases in humans or other animals. Mitochondria play a major role in iron metabolism.
与蛋白质和酶结合的铁，主要以血红素基团和铁硫簇的形式存在，对于氧气运输和许多其他生物学功能至关重要。全身铁稳态本质上是一个封闭系统。不存在铁排泄的调节机制，例如通过肝脏或肾脏排泄：铁的流失仅通过出血以及黏膜和皮肤细胞的脱落发生。这些流失通过肠道吸收得到补偿。相比之下，铁的吸收受到严格调节。最近发现的三种蛋白质，即HFE、铁调素和血色素沉着蛋白，在这种调节中起重要作用。另外大约20种蛋白质已被证明参与铁代谢，这通常是通过对人类或其他动物的遗传疾病的研究得出的。线粒体在铁代谢中起主要作用。
