The Serine Protease Matriptase-2 (TMPRSS6) Inhibits Hepcidin Activation by Cleaving Membrane Hemojuvelin
نویسندگان
چکیده
منابع مشابه
Membrane-bound serine protease matriptase-2 (Tmprss6) is an essential regulator of iron homeostasis.
Proteolytic events at the cell surface are essential in the regulation of signal transduction pathways. During the past years, the family of type II transmembrane serine proteases (TTSPs) has acquired an increasing relevance because of their privileged localization at the cell surface, although our current understanding of the biologic function of most TTSPs is limited. Here we show that matrip...
متن کاملInto the matrix: regulation of the iron regulatory hormone hepcidin by matriptase-2.
Matriptase-2 is a recently identified membrane-bound, cell-surface serine protease expressed primarily in liver. Mutations in matriptase-2 in mice and humans cause iron-deficiency anemia that responds poorly to iron therapy. The poor response results from an inability to decrease hepcidin production during iron deficiency. Cell culture studies reveal that matriptase-2 inhibits hepcidin inductio...
متن کاملIron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.
Hepcidin is the master regulator of iron homeostasis. In the liver, iron-dependent hepcidin activation is regulated through Bmp6 and its membrane receptor hemojuvelin (Hjv), whereas, in response to iron deficiency, hepcidin repression seems to be controlled by a pathway involving the serine protease matriptase-2 (encoded by Tmprss6). To determine the relationship between Bmp6 and matriptase-2 p...
متن کاملRetinal expression of the serine protease matriptase-2 (Tmprss6) and its role in retinal iron homeostasis
PURPOSE Matriptase-2 (also known as TMPRSS6) is a critical regulator of the iron-regulatory hormone hepcidin in the liver; matriptase-2 cleaves membrane-bound hemojuvelin and consequently alters bone morphogenetic protein (BMP) signaling. Hemojuvelin and hepcidin are expressed in the retina and play a critical role in retinal iron homeostasis. However, no information on the expression and funct...
متن کاملRole of matriptase-2 (TMPRSS6) in iron metabolism.
Iron, an essential element for life, is regulated primarily at the level of uptake, storage, and transport in order to maintain sufficient availability for normal physiology. The key protein in iron homeostasis is a 25-amino-acid peptide, hepcidin, which modulates the amount of iron in the circulation by binding and promoting the degradation of the iron exporter ferroportin. Given the central i...
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ژورنال
عنوان ژورنال: Cell Metabolism
سال: 2008
ISSN: 1550-4131
DOI: 10.1016/j.cmet.2008.09.012