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

Blood Cells, Molecules, and Diseases

Volumn 47, Issue 2, 2011, Pages 133-137

  Krijt, Jan ^a   Fujikura, Yuzo ^a   Ramsay, Andrew J ^b   Velasco, Gloria ^b   Nečas, Emanuel ^a  

^a CHARLES UNIVERSITY   (Czech Republic)

^b DEPARTAMENTO DE BIOQUÍMICA Y BIOLOGÍA MOLECULAR   (Spain)

Author keywords

Hemojuvelin; Hepcidin; Iron metabolism; TMPRSS6

Indexed keywords

GLYCOSYLPHOSPHATIDYLINOSITOL; HEMOJUVELIN; HEPCIDIN; HETERODIMER; MATRIPTASE; MATRIPTASE 2; PHOSPHATIDYLINOSITOL DIACYLGLYCEROL LYASE; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; ENZYME REGULATION; GENE; IN VIVO STUDY; IRON DEFICIENCY ANEMIA; LIVER HOMOGENATE; LIVER MEMBRANE; MALE; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN BLOOD LEVEL; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN PROTEIN INTERACTION; PROTEIN SYNTHESIS; TMPRSS6 GENE; WILD TYPE;

ANEMIA, IRON-DEFICIENCY; ANIMALS; ANTIMICROBIAL CATIONIC PEPTIDES; CELL MEMBRANE; DIMERIZATION; ELECTROPHORESIS, POLYACRYLAMIDE GEL; GENE EXPRESSION REGULATION; GLYCOSYLPHOSPHATIDYLINOSITOLS; IRON; LIVER; MALE; MEMBRANE PROTEINS; MICE; MICE, KNOCKOUT; PHOSPHOINOSITIDE PHOSPHOLIPASE C; POLYMERASE CHAIN REACTION; SERINE ENDOPEPTIDASES; SIGNAL TRANSDUCTION; TISSUE EXTRACTS;

MUS;

EID: 79960848290     PISSN: 10799796     EISSN: 10960961     Source Type: Journal    
DOI: 10.1016/j.bcmd.2011.04.009     Document Type: Article

References (25)

1. Nemeth E., Ganz T. The role of hepcidin in iron metabolism. Acta Haematol. 2009, 122:78-86.
2. Andrews N.C. Forging a field: the golden age of iron biology. Blood 2008, 112:219-230.
3. Hentze M.W., Muckenthaler M.U., Galy B., Camaschella C. Two to tango: regulation of mammalian iron metabolism. Cell 2010, 142:24-38.
4. Babitt J.L., Huang F.W., Wrighting D.M., Xia Y., Sidis Y., Samad T.A., Campagna J.A., Chung R.T., Schneyer A.L., Woolf C.J., Andrews N.C., Lin H.Y. Bone morphogenetic protein signaling by hemojuvelin regulates hepcidin expression. Nat. Genet. 2006, 38:531-539.
5. Papanikolaou G., Samuels M.E., Ludwig E.H., MacDonald M.L., Franchini P.L., Dubé M.P., Andres L., MacFarlane J., Sakellaropoulos N., Politou M., Nemeth E., Thompson J., Risler J.K., Zaborowska C., Babakaiff R., Radomski C.C., Pape T.D., Davidas O., Christakis J., Brissot P., Lockitch G., Ganz T., Hayden M.R., Goldberg Y.P. Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat. Genet. 2004, 36:77-82.
6. Du X., She E., Gelbart T., Truksa J., Lee P., Xia Y., Khovananth K., Mudd S., Mann N., Moresco E.M., Beutler E., Beutler B. The serine protease TMPRSS6 is required to sense iron deficiency. Science 2008, 320:1088-1092.
7. Finberg K.E., Heeney M.M., Campagna D.R., Aydinok Y., Pearson H.A., Hartman K.R., Mayo M.M., Samuel S.M., Strouse J.J., Markianos K., Andrews N.C., Fleming M.D. Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA). Nat. Genet. 2008, 40:569-571.
8. Folgueras A.R., de Lara F.M., Pendás A.M., Garabaya C., Rodríguez F., Astudillo A., Bernal T., Cabanillas R., López-Otín C., Velasco G. Membrane-bound serine protease matriptase-2 (Tmprss6) is an essential regulator of iron homeostasis. Blood 2008, 112:2539-2545.
9. Silvestri L., Pagani A., Nai A., De Domenico I., Kaplan J., Camaschella C. The serine protease matriptase-2 (TMPRSS6) inhibits hepcidin activation by cleaving membrane hemojuvelin. Cell Metab. 2008, 8:502-511.
10. Maxson J., Chen J., Enns C.A., Zhang A.S. Matriptase-2 and proprotein convertase-cleaved forms of hemojuvelin have different roles in the down-regulation of hepcidin expression. J. Biol. Chem. 2010, 285:39021-39028.
11. Truksa J., Gelbart T., Peng H., Beutler E., Beutler B., Lee P. Suppression of the hepcidin-encoding gene Hamp permits iron overload in mice lacking both hemojuvelin and matriptase-2/TMPRSS6. Br. J. Haematol. 2009, 147:571-581.
12. Finberg K.E., Whittlesey R.L., Fleming M.D., Andrews N.C. Downregulation of Bmp/Smad signaling by Tmprss6 is required for maintenance of systemic iron homeostasis. Blood 2010, 115:3817-3826.
13. Peng H., Truksa J., Lee P. EPO-mediated reduction in Hamp expression in vivo corrects iron deficiency anaemia in TMPRSS6 deficiency. Br. J. Haematol. 2010, 151:106-109.
14. Niederkofler V., Salie R., Arber S. Hemojuvelin is essential for dietary iron sensing, and its mutation leads to severe iron overload. J. Clin. Invest. 2005, 115:2180-2186.
15. Rodriguez Martinez A., Niemelä O., Parkkila S. Hepatic and extrahepatic expression of the new iron regulatory protein hemojuvelin. Haematologica 2004, 89:1441-1445.
16. Lin L., Goldberg Y.P., Ganz T. Competitive regulation of hepcidin mRNA by soluble and cell-associated hemojuvelin. Blood 2005, 106:2884-2889.
17. Huang F.W., Pinkus J.L., Pinkus G.S., Fleming M.D., Andrews N.C. A mouse model of juvenile hemochromatosis. J. Clin. Invest. 2005, 115:2187-2191.
18. Zhang A.S., West A.P., Wyman A.E., Bjorkman P.J., Enns C.A. Interaction of hemojuvelin with neogenin results in iron accumulation in human embryonic kidney 293 cells. J. Biol. Chem. 2005, 280:33885-33894.
19. Kuninger D., Kuns-Hashimoto R., Kuzmickas R., Rotwein P. Complex biosynthesis of the muscle-enriched iron regulator RGMc. J. Cell Sci. 2006, 119:3273-3283.
20. Lin L., Nemeth E., Goodnough J.B., Thapa D.R., Gabayan V., Ganz T. Soluble hemojuvelin is released by proprotein convertase-mediated cleavage at a conserved polybasic RNRR site. Blood Cells Mol. Dis. 2008, 40:122-131.
21. Zhang A.S., Anderson S.A., Meyers K.R., Hernandez C., Eisenstein R.S., Enns C.A. Evidence that inhibition of hemojuvelin shedding in response to iron is mediated through neogenin. J. Biol. Chem. 2007, 282:12547-12556.
22. Zhang A.S., Yang F., Wang J., Tsukamoto H., Enns C.A. Hemojuvelin-neogenin interaction is required for bone morphogenic protein-4-induced hepcidin expression. J. Biol. Chem. 2009, 284:22580-22589.
23. Silvestri L., Pagani A., Fazi C., Gerardi G., Levi S., Arosio P., Camaschella C. Defective targeting of hemojuvelin to plasma membrane is a common pathogenetic mechanism in juvenile hemochromatosis. Blood 2007, 109:4503-4510.
24. Zhang A.S., Anderson S.A., Wang J., Yang F., DeMaster K., Ahmed R., Nizzi C.P., Eisenstein R.S., Tsukamoto H., Enns C.A. Suppression of hepatic hepcidin expression in response to acute iron deprivation is associated with an increase of matriptase-2 protein. Blood 2011, 117:1687-1699.
25. Zhang A.S., Gao J., Koeberl D.D., Enns C.A. The role of hepatocyte hemojuvelin in the regulation of bone morphogenic protein-6 and hepcidin expression in vivo. J. Biol. Chem. 2010, 285:16416-16423.