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用于系统性铁代谢及其紊乱研究的小鼠突变体:近期进展的最新情况

Murine mutants in the study of systemic iron metabolism and its disorders: an update on recent advances.

作者信息

Bartnikas Thomas B, Fleming Mark D, Schmidt Paul J

机构信息

Department of Pathology, Children's Hospital Boston, MA, USA.

出版信息

Biochim Biophys Acta. 2012 Sep;1823(9):1444-50. doi: 10.1016/j.bbamcr.2012.01.011. Epub 2012 Jan 28.

Abstract

Many past and recent advances in the field of iron metabolism have relied upon the use of mouse models of disease. These models have arisen spontaneously in breeder colonies or have been engineered for global or conditional ablation or overexpression of select genes. Full phenotypic characterization of these models typically involves maintenance on iron-loaded or -deficient diets, treatment with oxidative or hemolytic agents, breeding to other mutant lines or other stresses. In this review, we focus on systemic iron biology and the contributions that mouse model-based studies have made to the field. We have divided the field into three broad areas of research: dietary iron absorption, regulation of hepcidin expression and cellular iron metabolism. For each area, we begin with an overview of the current understanding of key molecular and cellular determinants then discuss recent advances. Finally, we conclude with brief comments on prospects for future study. This article is part of a Special Issue entitled: Cell Biology of Metals.

摘要

铁代谢领域过去和近期的许多进展都依赖于疾病小鼠模型的使用。这些模型在繁殖群体中自发出现,或者经过基因工程改造,以实现特定基因的全身或条件性敲除或过表达。这些模型的全面表型特征通常涉及在铁负荷或铁缺乏饮食条件下饲养、用氧化或溶血剂处理、与其他突变系杂交或施加其他应激。在本综述中,我们聚焦于全身铁生物学以及基于小鼠模型的研究对该领域的贡献。我们将该领域分为三个广泛的研究领域:膳食铁吸收、铁调素表达的调节和细胞铁代谢。对于每个领域,我们首先概述当前对关键分子和细胞决定因素的理解,然后讨论近期进展。最后,我们对未来研究的前景进行简要评论。本文是名为《金属细胞生物学》特刊的一部分。

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