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Essential role of endocytosis of the type II transmembrane serine protease TMPRSS6 in regulating its functionality.

Béliveau F, Brulé C, Désilets A, Zimmerman B, Laporte SA, Lavoie CL, Leduc R.

J Biol Chem. 2011 Aug 19;286(33):29035-43. doi: 10.1074/jbc.M111.223461. Epub 2011 Jul 1.


Regulation of type II transmembrane serine proteinase TMPRSS6 by hypoxia-inducible factors: new link between hypoxia signaling and iron homeostasis.

Lakhal S, Schödel J, Townsend AR, Pugh CW, Ratcliffe PJ, Mole DR.

J Biol Chem. 2011 Feb 11;286(6):4090-7. doi: 10.1074/jbc.M110.173096. Epub 2010 Oct 21.


Retinal expression of the serine protease matriptase-2 (Tmprss6) and its role in retinal iron homeostasis.

Gnana-Prakasam JP, Baldowski RB, Ananth S, Martin PM, Smith SB, Ganapathy V.

Mol Vis. 2014 Apr 26;20:561-74. eCollection 2014.


The serine protease matriptase-2 (TMPRSS6) inhibits hepcidin activation by cleaving membrane hemojuvelin.

Silvestri L, Pagani A, Nai A, De Domenico I, Kaplan J, Camaschella C.

Cell Metab. 2008 Dec;8(6):502-11. doi: 10.1016/j.cmet.2008.09.012. Epub 2008 Oct 30.


A mutation in the TMPRSS6 gene, encoding a transmembrane serine protease that suppresses hepcidin production, in familial iron deficiency anemia refractory to oral iron.

Melis MA, Cau M, Congiu R, Sole G, Barella S, Cao A, Westerman M, Cazzola M, Galanello R.

Haematologica. 2008 Oct;93(10):1473-9. doi: 10.3324/haematol.13342. Epub 2008 Jul 4.


Identification of TMPRSS6 cleavage sites of hemojuvelin.

Rausa M, Ghitti M, Pagani A, Nai A, Campanella A, Musco G, Camaschella C, Silvestri L.

J Cell Mol Med. 2015 Apr;19(4):879-88. doi: 10.1111/jcmm.12462. Epub 2015 Feb 22.


Functional analysis of matriptase-2 mutations and domains: insights into the molecular basis of iron-refractory iron deficiency anemia.

McDonald CJ, Ostini L, Bennett N, Subramaniam N, Hooper J, Velasco G, Wallace DF, Subramaniam VN.

Am J Physiol Cell Physiol. 2015 Apr 1;308(7):C539-47. doi: 10.1152/ajpcell.00264.2014. Epub 2015 Jan 14.


Liver hemojuvelin protein levels in mice deficient in matriptase-2 (Tmprss6).

Krijt J, Fujikura Y, Ramsay AJ, Velasco G, Nečas E.

Blood Cells Mol Dis. 2011 Aug 15;47(2):133-7. doi: 10.1016/j.bcmd.2011.04.009. Epub 2011 May 25.


Down-regulation of Bmp/Smad signaling by Tmprss6 is required for maintenance of systemic iron homeostasis.

Finberg KE, Whittlesey RL, Fleming MD, Andrews NC.

Blood. 2010 May 6;115(18):3817-26. doi: 10.1182/blood-2009-05-224808. Epub 2010 Mar 3.


Regulation of TMPRSS6 by BMP6 and iron in human cells and mice.

Meynard D, Vaja V, Sun CC, Corradini E, Chen S, López-Otín C, Grgurevic L, Hong CC, Stirnberg M, Gütschow M, Vukicevic S, Babitt JL, Lin HY.

Blood. 2011 Jul 21;118(3):747-56. doi: 10.1182/blood-2011-04-348698. Epub 2011 May 26.


Neogenin interacts with matriptase-2 to facilitate hemojuvelin cleavage.

Enns CA, Ahmed R, Zhang AS.

J Biol Chem. 2012 Oct 12;287(42):35104-17. doi: 10.1074/jbc.M112.363937. Epub 2012 Aug 14.


Role of matriptase-2 (TMPRSS6) in iron metabolism.

Lee P.

Acta Haematol. 2009;122(2-3):87-96. doi: 10.1159/000243792. Epub 2009 Nov 10. Review.


A novel TMPRSS6 mutation that prevents protease auto-activation causes IRIDA.

Altamura S, D'Alessio F, Selle B, Muckenthaler MU.

Biochem J. 2010 Nov 1;431(3):363-71. doi: 10.1042/BJ20100668.


Hepcidin downregulation by repeated bleeding is not mediated by soluble hemojuvelin.

Krijt J, Fujikura Y, Sefc L, Vokurka M, Hlobenová T, Necas E.

Physiol Res. 2010;59(1):53-9. Epub 2009 Feb 27.


TMPRSS6 rs855791 modulates hepcidin transcription in vitro and serum hepcidin levels in normal individuals.

Nai A, Pagani A, Silvestri L, Campostrini N, Corbella M, Girelli D, Traglia M, Toniolo D, Camaschella C.

Blood. 2011 Oct 20;118(16):4459-62. doi: 10.1182/blood-2011-06-364034. Epub 2011 Aug 26.


A novel mutation Gly603Arg of TMPRSS6 in a Korean female with iron-refractory iron deficiency anemia.

Choi HS, Yang HR, Song SH, Seo JY, Lee KO, Kim HJ.

Pediatr Blood Cancer. 2012 Apr;58(4):640-2. doi: 10.1002/pbc.23190. Epub 2011 May 25. Erratum in: Pediatr Blood Cancer. 2012 Dec 15;59(7):1336.


Matriptase-2- and proprotein convertase-cleaved forms of hemojuvelin have different roles in the down-regulation of hepcidin expression.

Maxson JE, Chen J, Enns CA, Zhang AS.

J Biol Chem. 2010 Dec 10;285(50):39021-8. doi: 10.1074/jbc.M110.183160. Epub 2010 Oct 11.


The serine protease TMPRSS6 is required to sense iron deficiency.

Du X, She E, Gelbart T, Truksa J, Lee P, Xia Y, Khovananth K, Mudd S, Mann N, Moresco EM, Beutler E, Beutler B.

Science. 2008 May 23;320(5879):1088-92. doi: 10.1126/science.1157121. Epub 2008 May 1.


Suppression of the hepcidin-encoding gene Hamp permits iron overload in mice lacking both hemojuvelin and matriptase-2/TMPRSS6.

Truksa J, Gelbart T, Peng H, Beutler E, Beutler B, Lee P.

Br J Haematol. 2009 Nov;147(4):571-81. doi: 10.1111/j.1365-2141.2009.07873.x. Epub 2009 Sep 8.


Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.

Lenoir A, Deschemin JC, Kautz L, Ramsay AJ, Roth MP, Lopez-Otin C, Vaulont S, Nicolas G.

Blood. 2011 Jan 13;117(2):647-50. doi: 10.1182/blood-2010-07-295147. Epub 2010 Oct 12.

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