| Transmembrane serine protease 6, a novel target for inhibition of neuronal tumor growth. | Transmembrane serine protease 6, a novel target for inhibition of neuronal tumor growth.
Zuo Y, Bai J, Bai H, Tian S, Sun H, Shi Z, Yu P, Gao G, Li Y, Chang YZ., Free PMC Article | 01/17/2024 |
| Matriptase-2 and Hemojuvelin in Hepcidin Regulation: In Vivo Immunoblot Studies in Mask Mice. | Matriptase-2 and Hemojuvelin in Hepcidin Regulation: In Vivo Immunoblot Studies in Mask Mice.
Krijt J, Frýdlová J, Gurieva I, Přikryl P, Báječný M, Steinbicker AU, Vokurka M, Truksa J., Free PMC Article | 05/1/2021 |
| Correcting beta-thalassemia by combined therapies that restrict iron and modulate erythropoietin activity. | Correcting β-thalassemia by combined therapies that restrict iron and modulate erythropoietin activity.
Casu C, Pettinato M, Liu A, Aghajan M, Lo Presti V, Lidonnici MR, Munoz KA, O'Hara E, Olivari V, Di Modica SM, Booten S, Guo S, Neil G, Miari R, Shapir N, Zafir-Lavie I, Domev H, Ferrari G, Sitara D, Nai A, Rivella S., Free PMC Article | 03/27/2021 |
| The ectodomain of matriptase-2 plays an important nonproteolytic role in suppressing hepcidin expression in mice. | The ectodomain of matriptase-2 plays an important nonproteolytic role in suppressing hepcidin expression in mice.
Enns CA, Jue S, Zhang AS., Free PMC Article | 03/20/2021 |
| Genetic loss of Tmprss6 alters terminal erythroid differentiation in a mouse model of beta-thalassemia intermedia. | Genetic loss of Tmprss6 alters terminal erythroid differentiation in a mouse model of β-thalassemia intermedia.
Stagg DB, Whittlesey RL, Li X, Lozovatsky L, Gardenghi S, Rivella S, Finberg KE., Free PMC Article | 07/11/2020 |
| our results highlight the importance of understanding the precise function of each TMPRSS6 isoforms both in human and in mouse | Transcriptome analysis reveals TMPRSS6 isoforms with distinct functionalities.
Dion SP, Béliveau F, Désilets A, Ghinet MG, Leduc R., Free PMC Article | 11/2/2019 |
| The study describes a role for C and hepcidin in obesity and highlights the relevance of iron regulation in the control of adipose tissue function. | Matriptase-2 deficiency protects from obesity by modulating iron homeostasis.
Folgueras AR, Freitas-Rodríguez S, Ramsay AJ, Garabaya C, Rodríguez F, Velasco G, López-Otín C., Free PMC Article | 11/24/2018 |
| Erythroferrone and matriptase-2 independently regulate hepcidin expression. | Erythroferrone and matriptase-2 independently regulate hepcidin expression.
Aschemeyer S, Gabayan V, Ganz T, Nemeth E, Kautz L., Free PMC Article | 12/2/2017 |
| Data (including data from studies using knockout mice) suggest that Mt2/Tmprss6 suppresses hepcidin expression in hepatocytes independently of Hjv; Mt2/Tmprss6 cleaves Alk2, Alk3, ActRIIA, Bmpr2, Hfe, and, to a lesser extent, Hjv and Tfr2; thus, Mt2/Tmprss6 suppresses hepcidin expression by cleaving multiple components of the hepcidin induction pathway. (Mt2/Tmprss6 = matriptase-2; Hjv = hemojuvelin) | Matriptase-2 suppresses hepcidin expression by cleaving multiple components of the hepcidin induction pathway.
Wahedi M, Wortham AM, Kleven MD, Zhao N, Jue S, Enns CA, Zhang AS., Free PMC Article | 11/25/2017 |
| The results provide support for the interaction between TMPRSS6 and hemojuvelin in vivo; they also suggest that hemojuvelin could be cleaved by another as yet unknown protease in the absence of functional TMPRSS6. | Effect of erythropoietin administration on proteins participating in iron homeostasis in Tmprss6-mutated mask mice.
Frýdlová J, Rychtarčíková Z, Gurieva I, Vokurka M, Truksa J, Krijt J., Free PMC Article | 11/4/2017 |
| Studies in colonic T84 cell monolayers revealed that barrier disruption by the colitis-associated Th2-type cytokines, IL-4 and IL-13, down-regulates matriptase as well as prostasin through phosphorylation of the transcriptional regulator STAT6 | Inflammatory cytokines down-regulate the barrier-protective prostasin-matriptase proteolytic cascade early in experimental colitis.
Buzza MS, Johnson TA, Conway GD, Martin EW, Mukhopadhyay S, Shea-Donohue T, Antalis TM., Free PMC Article | 07/8/2017 |
| the function of matriptase-2 is dominant over that of ERFE and is essential in facilitating hepcidin suppression by attenuating the BMP-SMAD signaling. | Limiting hepatic Bmp-Smad signaling by matriptase-2 is required for erythropoietin-mediated hepcidin suppression in mice.
Nai A, Rubio A, Campanella A, Gourbeyre O, Artuso I, Bordini J, Gineste A, Latour C, Besson-Fournier C, Lin HY, Coppin H, Roth MP, Camaschella C, Silvestri L, Meynard D., Free PMC Article | 06/24/2017 |
| role of fetuin-A in iron homeostasis and provide new insights into the mechanism of how matriptase-2 might modulate hepcidin expression | Cell surface serine protease matriptase-2 suppresses fetuin-A/AHSG-mediated induction of hepcidin.
Stirnberg M, Maurer E, Arenz K, Babler A, Jahnen-Dechent W, Gütschow M. | 08/15/2015 |
| TMPRSS6 inhibition via decreased STAT5 phosphorylation may be an additional mechanism by which inflammation stimulates hepcidin expression to regulate iron homeostasis and immunity. | Inflammation regulates TMPRSS6 expression via STAT5.
Meynard D, Sun CC, Wu Q, Chen W, Chen S, Nelson CN, Waters MJ, Babitt JL, Lin HY., Free PMC Article | 03/7/2015 |
| matriptase-2 (encoded by Tmprss6)-is responsible for hepcidin repression throughout development, with its deficiency leading to increased hepcidin levels triggering iron deficiency and anemia starting in utero | Matriptase-2 is essential for hepcidin repression during fetal life and postnatal development in mice to maintain iron homeostasis.
Willemetz A, Lenoir A, Deschemin JC, Lopez-Otin C, Ramsay AJ, Vaulont S, Nicolas G. | 10/11/2014 |
| The iron-regulatory serine protease matriptase-2 is expressed in the retina, and absence of this enzyme leads to iron deficiency. | 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., Free PMC Article | 10/4/2014 |
| Loss of matriptase-2 increases bone morphogenetic protein-dependent signaling, while paradoxically decreasing liver hemojuvelin protein content. | Decreased hemojuvelin protein levels in mask mice lacking matriptase-2-dependent proteolytic activity.
Frýdlová J, Fujikura Y, Vokurka M, Nečas E, Krijt J. | 04/5/2014 |
| Results confirm the anti-inflammatory status of Tmprss6 KO mice and identify new potential target pathways/genes of Tmprss6. | A strong anti-inflammatory signature revealed by liver transcription profiling of Tmprss6-/- mice.
Riba M, Rausa M, Sorosina M, Cittaro D, Garcia Manteiga JM, Nai A, Pagani A, Martinelli-Boneschi F, Stupka E, Camaschella C, Silvestri L., Free PMC Article | 03/29/2014 |
| Tmprss6 gene knockdown reduces iron overload in an animal model of hemochromatosis and improves both iron overload and anemia in mice affected by beta-thalassemia. | Reducing TMPRSS6 ameliorates hemochromatosis and β-thalassemia in mice.
Guo S, Casu C, Gardenghi S, Booten S, Aghajan M, Peralta R, Watt A, Freier S, Monia BP, Rivella S., Free PMC Article | 05/25/2013 |
| Double mutant mice lacking functional Hfe or Tfr2 and Tmprss6 exhibited a severe iron deficiency microcytic anemia phenotype mimicking the phenotype of single mutant mice lacking functional Tmprss6 demonstrating that Hfe and Tfr2 are not substrates for Tmprss6. | Severe microcytic anemia but increased erythropoiesis in mice lacking Hfe or Tfr2 and Tmprss6.
Lee P, Hsu MH, Welser-Alves J, Peng H., Free PMC Article | 11/24/2012 |
| Preventing iron overload improves beta-thalassemia and strengthens the essential role of Tmprss6 for Hamp suppression, providing a proof of concept that Tmprss6 manipulation can offer a novel therapeutic option in this condition. | Deletion of TMPRSS6 attenuates the phenotype in a mouse model of β-thalassemia.
Nai A, Pagani A, Mandelli G, Lidonnici MR, Silvestri L, Ferrari G, Camaschella C., Free PMC Article | 08/11/2012 |
| The data do not provide support for the concept that matriptase-2 cleaves membrane hemojuvelin and may indicate that, in vivo, the role of matriptase-2 in the regulation of hepcidin gene expression is more complex. | Liver hemojuvelin protein levels in mice deficient in matriptase-2 (Tmprss6).
Krijt J, Fujikura Y, Ramsay AJ, Velasco G, Nečas E. | 01/7/2012 |
| Heterozygous loss of Tmprss6 in Hfe(-/-) mice reduced systemic iron overload, whereas homozygous loss caused systemic iron deficiency and elevated hepatic expression of hepcidin and other Bmp/Smad target genes | Tmprss6 is a genetic modifier of the Hfe-hemochromatosis phenotype in mice.
Finberg KE, Whittlesey RL, Andrews NC., Free PMC Article | 07/2/2011 |
| Loss of TMPRSS6 is associated with iron-deficiency anemia and involves up-regulation of Bmp/Smad signaling. | Down-regulation of Bmp/Smad signaling by Tmprss6 is required for maintenance of systemic iron homeostasis.
Finberg KE, Whittlesey RL, Fleming MD, Andrews NC., Free PMC Article | 05/31/2010 |
| matriptase-2 activity represents a novel and relevant step in hepcidin regulation and iron homeostasis | Membrane-bound serine protease matriptase-2 (Tmprss6) is an essential regulator of iron homeostasis.
Folgueras AR, de Lara FM, Pendás AM, Garabaya C, Rodríguez F, Astudillo A, Bernal T, Cabanillas R, López-Otín C, Velasco G. | 01/21/2010 |