期刊
JOURNAL OF HEPATOLOGY
卷 64, 期 2, 页码 505-515出版社
ELSEVIER
DOI: 10.1016/j.jhep.2015.11.009
关键词
Iron; Transferrin; Ferritin; Hepcidin; Ceruloplasmin; Ferroportin; Erythroferrone; Non-transferrin bound iron; Hemojuvelin; Transferrin receptor; Haemochromatosis; Liver
资金
- EuroIron1 (European Community) [LSHM-CT-2006-037296]
- IronReg (French National Research Agency, France)
- Afemers
Body iron has a very close relationship with the liver. Physiologically, the liver synthesizes transferrin, in charge of blood iron transport; ceruloplasmin, acting through its ferroxidase activity; and hepcidin, the master regulator of systemic iron. It also stores iron inside ferritin and serves as an iron reservoir, both protecting the cell from free iron toxicity and ensuring iron delivery to the body whenever needed. The liver is first in line for receiving iron from the gut and the spleen, and is, therefore, highly exposed to iron overload when plasma iron is in excess, especially through its high affinity for plasma non-transferrin bound iron. The liver is strongly involved when iron excess is related either to hepcidin deficiency, as in HFE, hemojuvelin, hepcidin, and transferrin receptor 2 related haemochromatosis, or to hepcidin resistance, as in type B ferroportin disease. It is less involved in the usual (type A) form of ferroportin disease which targets primarily the macrophagic system. Hereditary aceruloplasminemia raises important pathophysiological issues in light of its peculiar organ iron distribution. (C) 2015 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据