The role of TMPRSS6/matriptase-2 in iron regulation and anemia

被引：51

作者：

Wang, Chia-Yu ^{[1
]}

Meynard, Delphine ^{[2
]}

Lin, Herbert Y. ^{[1
]}

机构：

[1] Harvard Univ, Massachusetts Gen Hosp, Sch Med,Program Anemia Signaling Res, Ctr Syst Biol,Div Nephrol,Program Membrane Biol, Boston, MA 02114 USA

[2] Univ Toulouse 3, CNRS, INSERM, Ctr Physiopathol Toulouse Purpan,U1043,U5282, F-31062 Toulouse, France

来源：

FRONTIERS IN PHARMACOLOGY | 2014年 / 5卷

基金：

美国国家卫生研究院;

关键词：

iron; TMPRSS6; matriptase-2; iron overload; IRIDA; TRANSMEMBRANE SERINE-PROTEASE; GENOME-WIDE ASSOCIATION; NEGATIVE BREAST-CANCER; BETA-THALASSEMIC MICE; CELLS IN-VITRO; HEPCIDIN EXPRESSION; DEFICIENCY ANEMIA; MATRIPTASE-2; TMPRSS6; TRANSFERRIN RECEPTOR; MICROCYTIC ANEMIA;

D O I：

10.3389/fphar.2014.00114

中图分类号：

R9 [药学];

学科分类号：

1007 ;

摘要：

Matriptase-2, encoded by the TMPRSS6 gene, is a member of the type II transmembrane serine protease family. Matriptase-2 has structural and enzymatic similarities to matriptase-1, which has been implicated in cancer progression. Matriptase-2 was later established to be essential in iron homeostasis based on the phenotypes of iron-refractory iron deficiency anemia identified in mouse models as well as in human patients with TMPRSS6 mutations. TMPRSS6 is expressed mainly in the liver and negatively regulates the production of hepcidin, the systemic iron regulatory hormone. This review focuses on the current understanding of matriptase-2 biochemistry, and its role in iron metabolism and cancer progression. In light of recent investigations, the function of matriptase-2 in hepcidin regulation, how it is being regulated, as well as the therapeutic potential of matriptase-2 are also discussed.

引用

页数：6

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