Literature for peptidase S01.308: matriptase-2

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Literature Substrates

(Topics flags: A Assay, M Mutation, P Specificity, I Inhibitor, K Knockout, V Review. To select only the references relevant to a single topic, click the link above. See explanation.)

2026

Enns,C.A., Jue,S. and Zhang,A.S. Matriptase-2-mediated suppression of hepatic hepcidin expression in mice requires hepatocyte neogenin J Biol Chem (2026) 302, 111142-111142. PubMed Europe PubMed DOI

2025

Zhou,Y.L., Wang,Z.X., Gao,J.Q., Xu,J., Qin,L., He,Y., Li,M. and He,J. Tmprss6 modulates radiation-induced liver injury through the hepcidin axis and PI3K/AKT pathway Int J Radiat Biol (2025) 101, 860-875. PubMed Europe PubMed DOI K

2024

Huang,D.W., He,G.Q., Guo,X., Wan,Z., Gao,J. and Yang,R. Iron-Refractory Iron Deficiency Anemia with Novel TMPRSS6 Mutation Indian J Pediatr (2024) 91, 311-311. PubMed Europe PubMed DOI
Zuo,Y., Bai,J., Bai,H., Tian,S., Sun,H., Shi,Z., Yu,P., Gao,G., Li,Y. and Chang,Y.Z. Transmembrane serine protease 6, a novel target for inhibition of neuronal tumor growth Cell Death Dis (2024) 15, 49-49. PubMed Europe PubMed DOI PMC EPMC

2023

Al-Jamea,L.H., Woodman,A., Heiba,M., Elshazly,S.A., Ben Khalaf,N., Al-Yami,F.S., Bilal Waheed,K., Al Mutair,A., Alsedi,A., Quiambao,J.V., Alzahrani,F.M., Albaqami,W.F., Al Qahtani,F.H., Mohammed Aljarah,N., Fathallah,D.M. and Halim Deifalla,A. TMPRSS6 gene mutations in six Saudi families with iron refractory iron deficiency anemia Gene (2023) 851, 146977-146977. PubMed Europe PubMed DOI
Wu,Q., Li,S., Zhang,X. and Dong,N. Type II Transmembrane Serine Proteases as Modulators in Adipose Tissue Phenotype and Function Biomedicines (2023) 11 PubMed Europe PubMed DOI PMC EPMC V

2022

Dion,S.P., Desilets,A., Lemieux,G. and Leduc,R. Functionally impaired isoforms regulate TMPRSS6 proteolytic activity PLoS ONE (2022) 17, e0273825-e0273825. PubMed Europe PubMed DOI PMC EPMC

2021

Schmidt,P.J., Fitzgerald,K., Butler,J.S. and Fleming,M.D. Global loss of Tfr2 with concomitant induced iron deficiency greatly ameliorates the phenotype of a murine thalassemia intermedia model Am J Hematol (2021) 96, 251-257. PubMed Europe PubMed DOI K
Vadolas,J., Ng,G.Z., Kysenius,K., Crouch,P.J., Dames,S., Eisermann,M., Nualkaew,T., Vilcassim,S., Schaeper,U. and Grigoriadis,G. SLN124, a GalNac-siRNA targeting transmembrane serine protease 6, in combination with deferiprone therapy reduces ineffective erythropoiesis and hepatic iron-overload in a mouse model of beta-thalassaemia Br J Haematol (2021) PubMed Europe PubMed DOI

2020

Belot,A., Gourbeyre,O., Palin,A., Rubio,A., Largounez,A., Besson-Fournier,C., Latour,C., Lorgouilloux,M., Gallitz,I., Montagner,A., Polizzi,A., Regnier,M., Smati,S., Zhang,A.S., Diaz-Munoz,M.D., Steinbicker,A.U., Guillou,H., Roth,M.P., Coppin,H. and Meynard,D. Endoplasmic reticulum stress controls iron metabolism through TMPRSS6 repression and hepcidin mRNA stabilization by RNA-binding protein HuR Haematologica (2020) PubMed Europe PubMed DOI
Cakmakli,S., Kaplan,C., Uzunoglu,M., Buyukbayram,M., Gorgulu,E., Zarif,N.O. and Keskin,E.Y. A novel homozygous nonsense mutation (p.Y78*) in TMPRSS6 gene causing iron-refractory iron deficiency anemia (IRIDA) in two siblings Turk J Pediatr (2020) 62, 103-108. PubMed Europe PubMed DOI M
Enns,C.A., Jue,S. and Zhang,A.S. The ectodomain of matriptase-2 plays an important non-proteolytic role in suppressing hepcidin expression in mice Blood (2020) PubMed Europe PubMed DOI
Paszti-Gere,E., Szombath,G., Gutschow,M., Steinmetzer,T. and Szekacs,A. 3-Amidinophenylalanine-derived matriptase inhibitors can modulate hepcidin production in vitro Naunyn Schmiedebergs Arch Pharmacol (2020) 393, 511-520. PubMed Europe PubMed DOI I

2019

Beliveau,F., Tarkar,A., Dion,S.P., Desilets,A., Ghinet,M.G., Boudreault,P.L., St-Georges,C., Marsault,E., Paone,D., Collins,J., Macphee,C.H., Campobasso,N., Groy,A., Cottom,J., Ouellette,M., Pope,A.J. and Leduc,R. Discovery and development of TMPRSS6 inhibitors modulating hepcidin levels in human hepatocytes Cell Chem Biol (2019) 26, 1559-1572. PubMed Europe PubMed DOI I
Damalanka,V.C. and Janetka,J.W. Recent progress on inhibitors of the type II transmembrane serine proteases, hepsin, matriptase and matriptase-2 Future Med Chem (2019) 11, 743-769. PubMed Europe PubMed DOI I
Mao,P., Wortham,A.M., Enns,C.A. and Zhang,A.S. The catalytic, stem, and transmembrane portions of matriptase-2 are required for suppressing the expression of the iron-regulatory hormone hepcidin J Biol Chem (2019) 294, 2060-2073. PubMed Europe PubMed DOI

2018

Dion,S.P., Beliveau,F., Desilets,A., Ghinet,M.G. and Leduc,R. Transcriptome analysis reveals TMPRSS6 isoforms with distinct functionalities J Cell Mol Med (2018) 22, 2498-2509. PubMed Europe PubMed DOI
Folgueras,A.R., Freitas-Rodriguez,S., Ramsay,A.J., Garabaya,C., Rodriguez,F., Velasco,G. and Lopez-Otin,C. Matriptase-2 deficiency protects from obesity by modulating iron homeostasis Nat Commun (2018) 9, 1350-1350. PubMed Europe PubMed DOI PMC EPMC M
Gitlin-Domagalska,A., Mangold,M., Debowski,D., Ptaszynska,N., Legowska,A., Gutschow,M. and Rolka,K. Matriptase-2: monitoring and inhibiting its proteolytic activity Future Med Chem (2018) 10, 2745-2761. PubMed Europe PubMed DOI I
Mangold,M., Gutschow,M. and Stirnberg,M. A short peptide inhibitor as an activity-based probe for matriptase-2 Pharmaceuticals (Basel) (2018) 11 PubMed Europe PubMed DOI I

2017

Gitlin-Domagalska,A., Debowski,D., Legowska,A., Stirnberg,M., Okonska,J., Gutschow,M. and Rolka,K. Design and chemical syntheses of potent matriptase-2 inhibitors based on trypsin inhibitor SFTI-1 isolated from sunflower seeds Biopolymers (2017) 108 PubMed Europe PubMed DOI I
Pinto,J., Nobre de Jesus,G., Palma Anselmo,M., Goncalves,L., Bras,D., Madeira Lopes,J., Meneses,J., Victorino,R. and Faustino,P. Iron refractory iron deficiency anemia in dizygotic twins due to a novel TMPRSS6 gene mutation in addition to polymorphisms associated with high susceptibility to develop ferropenic anemia J Investig Med High Impact Case Rep (2017) 5, 2324709617701776-2324709617701776. PubMed Europe PubMed DOI PMC EPMC M
St-Georges,C., Desilets,A., Beliveau,F., Ghinet,M., Dion,S.P., Colombo,E., Boudreault,P.L., Najmanovich,R.J., Leduc,R. and Marsault,E. Modulating the selectivity of matriptase-2 inhibitors with unnatural amino acids Eur J Med Chem (2017) 129, 110-123. PubMed Europe PubMed DOI I
Wahedi,M., Wortham,A.M., Kleven,M.D., Zhao,N., Jue,S., Enns,C.A. and Zhang,A.S. Matriptase-2 suppresses hepcidin expression by cleaving multiple components of the hepcidin induction pathway J Biol Chem (2017) 292, 18354-18371. PubMed Europe PubMed DOI

2016

Beckmann,A.M., Maurer,E., Lulsdorff,V., Wilms,A., Furtmann,N., Bajorath,J., Gutschow,M. and Stirnberg,M. En route to new therapeutic options for iron overload diseases: matriptase-2 as a target for Kunitz-type inhibitors Chembiochem (2016) 17, 595-604. PubMed Europe PubMed DOI
Beckmann,A.M., Glebov,K., Walter,J., Merkel,O., Mangold,M., Schmidt,F., Becker-Pauly,C., Gutschow,M. and Stirnberg,M. The intact Kunitz domain protects the amyloid precursor protein from being processed by matriptase-2 Biol Chem (2016) 397, 777-790. PubMed Europe PubMed DOI
Beckmann,A.M., Gilberg,E., Gattner,S., Huang,T.L., Vanden Eynde,J.J., Mayence,A., Bajorath,J., Stirnberg,M. and Gutschow,M. Evaluation of bisbenzamidines as inhibitors for matriptase-2 Bioorg Med Chem Lett (2016) 26, 3741-3745. PubMed Europe PubMed DOI I
Haussler,D., Mangold,M., Furtmann,N., Braune,A., Blaut,M., Bajorath,J., Stirnberg,M. and Gutschow,M. Back cover: Phosphono bisbenzguanidines as irreversible dipeptidomimetic inhibitors and activity-based probes of matriptase-2 (Chem. Eur. J. 25/2016) Chemistry (2016) 22, 8704- DOI I
Sal,E., Keskin,E.Y., Yenicesu,I., Bruno,M. and De Falco,L. Iron-refractory iron deficiency anemia (IRIDA) cases with 2 novel TMPRSS6 mutations Pediatr Hematol Oncol (2016) 33, 226-232. PubMed Europe PubMed DOI M

2015

Gitlin,A., Debowski,D., Karna,N., Legowska,A., Stirnberg,M., Gutschow,M. and Rolka,K. Inhibitors of matriptase-2 based on the trypsin inhibitor SFTI-1 Chembiochem (2015) 16, 1601-1607. PubMed Europe PubMed DOI
Jackle,F., Schmidt,F., Wichert,R., Arnold,P., Prox,J., Mangold,M., Ohler,A., Pietrzik,C.U., Koudelka,T., Tholey,A., Gutschow,M., Stirnberg,M. and Becker-Pauly,C. Metalloprotease meprin beta is activated by transmembrane serine protease matriptase-2 at the cell surface thereby enhancing APP shedding Biochem J (2015) 470, 91-103. PubMed Europe PubMed DOI
McDonald,C.J., Ostini,L., Bennett,N.C., Subramaniam,N., Hooper,J., Velasco,G., Wallace,D.F. and Subramaniam,V.N. Functional analysis of matriptase-2 mutations and domains: insights into the molecular basis of iron-refractory iron deficiency anemia Am J Physiol Cell Physiol (2015) 308, 539-547. PubMed Europe PubMed DOI M
Poggiali,E., Andreozzi,F., Nava,I., Consonni,D., Graziadei,G. and Cappellini,M.D. The role of TMPRSS6 polymorphisms in iron deficiency anemia partially responsive to oral iron treatment Am J Hematol (2015) 90, 306-309. PubMed Europe PubMed DOI M
Rausa,M., Ghitti,M., Pagani,A., Nai,A., Campanella,A., Musco,G., Camaschella,C. and Silvestri,L. Identification of TMPRSS6 cleavage sites of hemojuvelin J Cell Mol Med (2015) 19, 879-888. PubMed Europe PubMed DOI P
Schmidt,P.J., Racie,T., Westerman,M., Fitzgerald,K., Butler,J.S. and Fleming,M.D. Combination therapy with a Tmprss6 RNAi-therapeutic and the oral iron chelator deferiprone additively diminishes secondary iron overload in a mouse model of beta-thalassemia intermedia Am J Hematol (2015) 90, 310-313. PubMed Europe PubMed DOI K
Stirnberg,M., Maurer,E., Arenz,K., Babler,A., Jahnen-Dechent,W. and Gutschow,M. Cell surface serine protease matriptase-2 suppresses fetuin-A/AHSG-mediated induction of hepcidin Biol Chem (2015) 396, 81-93. PubMed Europe PubMed DOI

2014

Barre,O., Dufour,A., Eckhard,U., Kappelhoff,R., Beliveau,F., Leduc,R. and Overall,C.M. Cleavage specificity analysis of six type II transmembrane serine proteases (TTSPs) using PICS with proteome-derived peptide libraries PLoS ONE (2014) 9, e105984-e105984. PubMed Europe PubMed DOI PMC EPMC P
De Falco,L., Silvestri,L., Kannengiesser,C., Moran,E., Oudin,C., Rausa,M., Bruno,M., Aranda,J., Argiles,B., Yenicesu,I., Falcon-Rodriguez,M., Yilmaz-Keskin,E., Kocak,U., Beaumont,C., Camaschella,C., Iolascon,A., Grandchamp,B. and Sanchez,M. Functional and clinical impact of novel TMPRSS6 variants in iron-refractory iron-deficiency anemia patients and genotype-phenotype studies Hum Mutat (2014) 35, 1321-1329. PubMed Europe PubMed DOI M
Duchene,D., Colombo,E., Desilets,A., Boudreault,P.L., Leduc,R., Marsault,E. and Najmanovich,R. Analysis of subpocket selectivity and identification of potent selective inhibitors for matriptase and matriptase-2 J Med Chem (2014) 57, 10198-10204. PubMed Europe PubMed DOI I
Gnana-Prakasam,J.P., Baldowski,R.B., Ananth,S., Martin,P.M., Smith,S.B. and Ganapathy,V. Retinal expression of the serine protease matriptase-2 (tmprss6) and its role in retinal iron homeostasis Mol Vision (2014) 20, 561-574. PubMed Europe PubMed
Jiang,J., Yang,J., Feng,P., Zuo,B., Dong,N., Wu,Q. and He,Y. N-glycosylation is required for matriptase-2 autoactivation and ectodomain shedding J Biol Chem (2014) 289, 19500-19507. PubMed Europe PubMed DOI
Nai,A., Pellegrino,R.M., Rausa,M., Pagani,A., Boero,M., Silvestri,L., Saglio,G., Roetto,A. and Camaschella,C. The erythroid function of transferrin receptor 2 revealed by TMPRSS6 inactivation in different models of transferrin receptor 2 knockout mice Haematologica (2014) 99, 1016-1021. PubMed Europe PubMed DOI K
Wang,C.Y., Meynard,D. and Lin,H.Y. The role of TMPRSS6/matriptase-2 in iron regulation and anemia Front Pharmacol (2014) 5, 114-114. PubMed Europe PubMed DOI PMC EPMC V
Willemetz,A., Lenoir,A., Deschemin,J.C., Lopez-Otin,C., Ramsay,A.J., Vaulont,S. and Nicolas,G. Matriptase-2 is essential for hepcidin repression during fetal life and postnatal development in mice to maintain iron homeostasis Blood (2014) 124, 441-444. PubMed Europe PubMed DOI
Wysocka,M., Gruba,N., Miecznikowska,A., Popow-Stellmaszyk,J., Gutschow,M., Stirnberg,M., Furtmann,N., Bajorath,J., Lesner,A. and Rolka,K. Substrate specificity of human matriptase-2 Biochimie (2014) 97, 121-127. PubMed Europe PubMed DOI P

2013

Bartnikas,T.B., Steinbicker,A.U., Campagna,D.R., Blevins,S., Woodward,L.S., Herrera,C., Bloch,K.D., Justice,M.J. and Fleming,M.D. Identification and characterization of a novel murine allele of Tmprss6 Haematologica (2013) 98, 854-861. PubMed Europe PubMed DOI M
De Falco,L., Sanchez,M., Silvestri,L., Kannengiesser,C., Muckenthaler,M.U., Iolascon,A., Gouya,L., Camaschella,C. and Beaumont,C. Iron refractory iron deficiency anemia Haematologica (2013) 98, 845-853. PubMed Europe PubMed DOI PMC EPMC M
Guo,S., Casu,C., Gardenghi,S., Booten,S., Aghajan,M., Peralta,R., Watt,A., Freier,S., Monia,B.P. and Rivella,S. Reducing TMPRSS6 ameliorates hemochromatosis and beta-thalassemia in mice J Clin Invest (2013) 123, 1531-1541. PubMed Europe PubMed DOI PMC EPMC K
Jaspers,A., Caers,J., Le Gac,G., Ferec,C., Beguin,Y. and Fillet,G. A novel mutation in the CUB sequence of matriptase-2 (Tmprss6) is implicated in iron-resistant iron deficiency anaemia (IRIDA) Br J Haematol (2013) 160, 564-565. PubMed Europe PubMed DOI M
Maurer,E., Gutschow,M. and Stirnberg,M. Hepatocyte growth factor activator inhibitor type 2 (HAI-2) modulates hepcidin expression by inhibiting the cell surface protease matriptase-2 Biochem J (2013) 450, 583-593. PubMed Europe PubMed DOI
Roetto,A. and Porto,G. A novel mutation in the CUB sequence of matriptase-2 (TMPRSS6) is implicated in iron-resistant iron deficiency anaemia - response to Jaspers etal Br J Haematol (2013) 160, 566-567. PubMed Europe PubMed DOI M
Schmidt,P.J., Toudjarska,I., Sendamarai,A.K., Racie,T., Milstein,S., Bettencourt,B.R., Hettinger,J., Bumcrot,D. and Fleming,M.D. An RNAi therapeutic targeting Tmprss6 decreases iron overload in Hfe(-/-) mice and ameliorates anemia and iron overload in murine beta-thalassemia intermedia Blood (2013) 121, 1200-1208. PubMed Europe PubMed DOI K
Stirnberg,M. and Gutschow,M. Matriptase-2, a regulatory protease of iron homeostasis: possible substrates, cleavage sites and inhibitors Curr Pharm Des (2013) 19, 1052-1061. PubMed Europe PubMed
Tuhkanen,H., Hartikainen,J.M., Soini,Y., Velasco,G., Sironen,R., Nykopp,T.K., Kataja,V., Eskelinen,M., Kosma,V.M. and Mannermaa,A. Matriptase-2 gene (TMPRSS6) variants associate with breast cancer survival, and reduced expression is related to triple-negative breast cancer Int J Cancer (2013) 133, 2334-2340. PubMed Europe PubMed DOI M
Webb,S.L., Sanders,A.J., Mason,M.D. and Jiang,W.G. Matriptase-2 inhibits HECV motility and tubule formation in vitro and tumour angiogenesis in vivo Mol Cell Biochem (2013) 375, 207-217. PubMed Europe PubMed DOI

2012

Choi,H.S., Yang,H.R., Song,S.H., Seo,J.Y., Lee,K.O. and Kim,H.J. A novel mutation Gly603Arg of TMPRSS6 in a Korean female with iron-refractory iron deficiency anemia Pediatr Blood Cancer (2012) 58, 640-642. PubMed Europe PubMed DOI M
Dosa,S., Stirnberg,M., Lulsdorff,V., Haussler,D., Maurer,E. and Gutschow,M. Active site mapping of trypsin, thrombin and matriptase-2 by sulfamoyl benzamidines Bioorg Med Chem (2012) 20, 6489-6505. PubMed Europe PubMed DOI I
Guillem,F., Kannengiesser,C., Oudin,C., Lenoir,A., Matak,P., Donadieu,J., Isidor,B., Mechinaud,F., Aguilar-Martinez,P., Beaumont,C., Vaulont,S., Grandchamp,B. and Nicolas,G. Inactive matriptase-2 mutants found in IRIDA patients still repress hepcidin in a transfection assay despite having lost their serine protease activity Hum Mutat (2012) 33, 1388-1396. PubMed Europe PubMed DOI M
Maurer,E., Sisay,M.T., Stirnberg,M., Steinmetzer,T., Bajorath,J. and Gutschow,M. Insights into matriptase-2 substrate binding and inhibition mechanisms by analyzing active-site-mutated variants ChemMedChem (2012) 7, 68-72. PubMed Europe PubMed DOI
Ramsay,A.J., Kwarciak,A., Hooper,J.D., Lopez-Otin,C. and Velasco,G. Matriptase-2 [ISSN:978-0-12-407742-3] (2012) 3, 2975-2983. DOI

2011

Antalis,T.M., Bugge,T.H. and Wu,Q. Membrane-anchored serine proteases in health and disease Prog Mol Biol Transl Sci (2011) 99, 1-50. PubMed Europe PubMed DOI V
Beliveau,F., Brule,C., Desilets,A., Zimmerman,B., Laporte,S.A., Lavoie,C.L. and Leduc,R. Essential role of endocytosis of the type II transmembrane serine protease TMPRSS6 in regulating its functionality J Biol Chem (2011) 286, 29035-29043. PubMed Europe PubMed DOI
Finberg,K.E., Whittlesey,R.L. and Andrews,N.C. Tmprss6 is a genetic modifier of the Hfe-hemochromatosis phenotype in mice Blood (2011) 117, 4590-4599. PubMed Europe PubMed DOI M
Krijt,J., Fujikura,Y., Ramsay,A.J., Velasco,G. and Necas,E. Liver hemojuvelin protein levels in mice deficient in matriptase-2 (Tmprss6) Blood Cells Mol Dis (2011) 47, 133-137. PubMed Europe PubMed DOI
Lenoir,A., Deschemin,J.C., Kautz,L., Ramsay,A.J., Roth,M.P., Lopez-Otin,C., Vaulont,S. and Nicolas,G. Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6 Blood (2011) 117, 647-650. PubMed Europe PubMed DOI
Nai,A., Pagani,A., Silvestri,L., Campostrini,N., Corbella,M., Girelli,D., Traglia,M., Toniolo,D. and Camaschella,C. TMPRSS6 rs855791 modulates hepcidin transcription in vitro and serum hepcidin levels in normal individuals Blood (2011) 118, 4459-4462. PubMed Europe PubMed DOI
Sato,T., Iyama,S., Murase,K., Kamihara,Y., Ono,K., Kikuchi,S., Takada,K., Miyanishi,K., Sato,Y., Takimoto,R., Kobune,M. and Kato,J. Novel missense mutation in the TMPRSS6 gene in a Japanese female with iron-refractory iron deficiency anemia Int J Hematol (2011) 94, 101-103. PubMed Europe PubMed DOI M
Webb,S.L., Sanders,A.J., Mason,M.D. and Jiang,W.G. Type II transmembrane serine protease (TTSP) deregulation in cancer Front Biosci (2011) 16, 539-552. PubMed Europe PubMed V

2010

Altamura,S., D'Alessio,F., Selle,B. and Muckenthaler,M.U. A novel TMPRSS6 mutation that prevents protease auto-activation causes IRIDA Biochem J (2010) 431, 363-371. PubMed Europe PubMed DOI M
Cuijpers,M.L., Wiegerinck,E.T., Brouwer,R., de Witte,T.J. and Swinkels,D.W. [Iron deficiency anaemia due to a matriptase-2 mutation] Ned Tijdschr Geneeskd (2010) 154, A1038-A1038. PubMed Europe PubMed M
De Falco,L., Totaro,F., Nai,A., Pagani,A., Girelli,D., Silvestri,L., Piscopo,C., Campostrini,N., Dufour,C., Al Manjomi,F., Minkov,M., Van Vuurden,D.G., Feliu,A., Kattamis,A., Camaschella,C. and Iolascon,A. Novel TMPRSS6 mutations associated with iron-refractory iron deficiency anemia (IRIDA) Hum Mutat (2010) 31, E1390-E1405. PubMed Europe PubMed DOI M
Finberg,K.E., Whittlesey,R.L., Fleming,M.D. and Andrews,N.C. Down-regulation of Bmp/Smad signaling by Tmprss6 is required for maintenance of systemic iron homeostasis Blood (2010) 115, 3817-3826. PubMed Europe PubMed DOI M
Sanders,A.J., Webb,S.L., Parr,C., Mason,M.D. and Jiang,W.G. The type II transmembrane serine protease, matriptase-2: possible links to cancer? Anticancer Agents Med Chem (2010) 10, 64-69. PubMed Europe PubMed
Sisay,M.T., Steinmetzer,T., Stirnberg,M., Maurer,E., Hammami,M., Bajorath,J. and Gutschow,M. Identification of the first low-molecular-weight inhibitors of matriptase-2 J Med Chem (2010) 53, 5523-5535. PubMed Europe PubMed DOI I
Stirnberg,M., Maurer,E., Horstmeyer,A., Kolp,S., Frank,S., Bald,T., Arenz,K., Janzer,A., Prager,K., Wunderlich,P., Walter,J. and Gutschow,M. Proteolytic processing of the serine protease matriptase-2: identification of the cleavage sites required for its autocatalytic release from the cell surface Biochem J (2010) 430, 87-95. PubMed Europe PubMed DOI
Tanaka,T., Roy,C.N., Yao,W., Matteini,A., Semba,R.D., Arking,D., Walston,J.D., Fried,L.P., Singleton,A., Guralnik,J., Abecasis,G.R., Bandinelli,S., Longo,D.L. and Ferrucci,L. A genome-wide association analysis of serum iron concentrations Blood (2010) 115, 94-96. PubMed Europe PubMed DOI

2009

Beliveau,F., Desilets,A. and Leduc,R. Probing the substrate specificities of matriptase, matriptase-2, hepsin and DESC1 with internally quenched fluorescent peptides FEBS J (2009) 276, 2213-2226. PubMed Europe PubMed DOI A P
Benyamin,B., Ferreira,M.A., Willemsen,G., Gordon,S., Middelberg,R.P., McEvoy,B.P., Hottenga,J.J., Henders,A.K., Campbell,M.J., Wallace,L., Frazer,I.H., Heath,A.C., de Geus,E.J., Nyholt,D.R., Visscher,P.M., Penninx,B.W., Boomsma,D.I., Martin,N.G., Montgomery,G.W. and Whitfield,J.B. Common variants in TMPRSS6 are associated with iron status and erythrocyte volume Nat Genet (2009) 41, 1173-1175. PubMed Europe PubMed DOI PMC EPMC M
Chambers,J.C., Zhang,W., Li,Y., Sehmi,J., Wass,M.N., Zabaneh,D., Hoggart,C., Bayele,H., McCarthy,M.I., Peltonen,L., Freimer,N.B., Srai,S.K., Maxwell,P.H., Sternberg,M.J., Ruokonen,A., Abecasis,G., Jarvelin,M.R., Scott,J., Elliott,P. and Kooner,J.S. Genome-wide association study identifies variants in TMPRSS6 associated with hemoglobin levels Nat Genet (2009) 41, 1170-1172. PubMed Europe PubMed DOI
Cui,Y., Wu,Q. and Zhou,Y. Iron-refractory iron deficiency anemia: new molecular mechanisms Kidney Int (2009) 76, 1137-1141. PubMed Europe PubMed DOI
Edison,E.S., Athiyarath,R., Rajasekar,T., Westerman,M., Srivastava,A. and Chandy,M. A novel splice site mutation c.2278 (-1) G>C in the TMPRSS6 gene causes deletion of the substrate binding site of the serine protease resulting in refractory iron deficiency anaemia Br J Haematol (2009) 147, 766-769. PubMed Europe PubMed DOI M
Knutson,M.D. Into the matrix: regulation of the iron regulatory hormone hepcidin by matriptase-2 Nutr Rev (2009) 67, 284-288. PubMed Europe PubMed DOI
Ramsay,A.J., Quesada,V., Sanchez,M., Garabaya,C., Sarda,M.P., Baiget,M., Remacha,A., Velasco,G. and Lopez-Otin,C. Matriptase-2 mutations in iron-refractory iron deficiency anemia patients provide new insights into protease activation mechanisms Hum Mol Genet (2009) 18, 3673-3683. PubMed Europe PubMed DOI M
Ramsay,A.J., Hooper,J.D., Folgueras,A.R., Velasco,G. and Lopez-Otin,C. Matriptase-2 (TMPRSS6): a proteolytic regulator of iron homeostasis Haematologica (2009) 94, 840-849. PubMed Europe PubMed DOI PMC EPMC M
Silvestri,L., Guillem,F., Pagani,A., Nai,A., Oudin,C., Silva,M., Toutain,F., Kannengiesser,C., Beaumont,C., Camaschella,C. and Grandchamp,B. Molecular mechanisms of the defective hepcidin inhibition in TMPRSS6 mutations associated with iron-refractory iron deficiency anemia Blood (2009) 113, 5605-5608. PubMed Europe PubMed DOI M
Tchou,I., Diepold,M., Pilotto,P.A., Swinkels,D., Neerman-Arbez,M. and Beris,P. Haematologic data, iron parameters and molecular findings in two new cases of iron-refractory iron deficiency anaemia Eur J Haematol (2009) 83, 595-602. PubMed Europe PubMed DOI M
Truksa,J., Gelbart,T., Peng,H., Beutler,E., Beutler,B. and 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. PubMed Europe PubMed DOI

2008

Du,X., She,E., Gelbart,T., Truksa,J., Lee,P., Xia,Y., Khovananth,K., Mudd,S., Mann,N., Moresco,E.M., Beutler,E. and Beutler,B. The serine protease TMPRSS6 is required to sense iron deficiency Science (2008) 320, 1088-1092. PubMed Europe PubMed DOI PMC EPMC M
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. and Fleming,M.D. Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA) Nat Genet (2008) 40, 569-571. PubMed Europe PubMed DOI M
Folgueras,A.R., de Lara,F.M., Pendas,A.M., Garabaya,C., Rodriguez,F., Astudillo,A., Bernal,T., Cabanillas,R., Lopez-Otin,C. and Velasco,G. Membrane-bound serine protease matriptase-2 (Tmprss6) is an essential regulator of iron homeostasis Blood (2008) 112, 2539-2545. PubMed Europe PubMed DOI
Guillem,F., Lawson,S., Kannengiesser,C., Westerman,M., Beaumont,C. and Grandchamp,B. Two nonsense mutations in the TMPRSS6 gene in a patient with microcytic anemia and iron deficiency Blood (2008) 112, 2089-2091. PubMed Europe PubMed DOI M
Melis,M.A., Cau,M., Congiu,R., Sole,G., Barella,S., Cao,A., Westerman,M., Cazzola,M. and Galanello,R. A mutation in the TMPRSS6 gene, encoding a transmembrane serine protease that suppresses hepcidin production, in familial iron deficiency anemia refractory to oral iron Haematologica (2008) 93, 1473-1479. PubMed Europe PubMed DOI M
Muckenthaler,M.U. Fine tuning of hepcidin expression by positive and negative regulators Cell Metab (2008) 8, 1-3. PubMed Europe PubMed DOI
Ramsay,A.J., Reid,J.C., Velasco,G., Quigley,J.P. and Hooper,J.D. The type II transmembrane serine protease matriptase-2 - identification, structural features, enzymology, expression pattern and potential roles Front Biosci (2008) 13, 569-579. PubMed Europe PubMed DOI V
Silvestri,L., Pagani,A., Nai,A., De Domenico,I., Kaplan,J. and Camaschella,C. The serine protease matriptase-2 (TMPRSS6) inhibits hepcidin activation by cleaving membrane hemojuvelin Cell Metab (2008) 8, 502-511. PubMed Europe PubMed DOI PMC EPMC

2005

[YEAR:30-4-2005]Park,T.J., Lee,Y.J., Kim,H.J., Park,H.G. and Park,W.J. Cloning and characterization of TMPRSS6, a novel type 2 transmembrane serine protease Mol Cells (30-4-2005) 19, 223-227. PubMed Europe PubMed

2003

[YEAR:1-8-2003]Hooper,J.D., Campagnolo,L., Goodarzi,G., Truong,T.N., Stuhlmann,H. and Quigley,J.P. Mouse matriptase-2: identification, characterization and comparative mRNA expression analysis with mouse hepsin in adult and embryonic tissues Biochem J (1-8-2003) 373, 689-702. PubMed Europe PubMed DOI PMC EPMC
Szabo,R., Wu,Q., Dickson,R.B., Netzel-Arnett,S., Antalis,T.M. and Bugge,T.H. Type II transmembrane serine proteases Thromb Haemost (2003) 90, 185-193. PubMed Europe PubMed DOI V