The liver and muscle secreted HFE2-protein maintains central nervous system blood vessel integrity. | The liver and muscle secreted HFE2-protein maintains central nervous system blood vessel integrity. Wang XF, Vigouroux R, Syonov M, Baglaenko Y, Nikolakopoulou AM, Ringuette D, Rus H, DiStefano PV, Dufour S, Shabanzadeh AP, Lee S, Mueller BK, Charish J, Harada H, Fish JE, Wither J, Wälchli T, Cloutier JF, Zlokovic BV, Carlen PL, Monnier PP., Free PMC Article | 02/7/2024 |
Genetic Iron Overload Hampers Development of Cutaneous Leishmaniasis in Mice. | Genetic Iron Overload Hampers Development of Cutaneous Leishmaniasis in Mice. Charlebois E, Li Y, Wagner V, Pantopoulos K, Olivier M., Free PMC Article | 02/4/2023 |
Hepatocyte neogenin is required for hemojuvelin-mediated hepcidin expression and iron homeostasis in mice. | Hepatocyte neogenin is required for hemojuvelin-mediated hepcidin expression and iron homeostasis in mice. Enns CA, Jue S, Zhang AS., Free PMC Article | 12/4/2021 |
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 |
The functional role of hemojuvelin in acute ischemic stroke. | The functional role of hemojuvelin in acute ischemic stroke. Young GH, Tang SC, Wu VC, Wang KC, Nong JY, Huang PY, Hu CJ, Chiou HY, Jeng JS, Hsu CY., Free PMC Article | 01/9/2021 |
Hjv was significantly downregulated in the muscles of mdx mice as well as in those of aged mice. Overexpression of Hjv rescued dystrophic and age-related muscle wasting. Mechanistically, loss of Hjv promoted activation of Smad3 signalling induced by TGF-beta1, whereas Hjv overexpression inhibited TGF-beta1/Smad3 signalling by directly interacting with TbetaRII on the muscle membrane. | Hemojuvelin is a novel suppressor for Duchenne muscular dystrophy and age-related muscle wasting. Zhang P, He J, Wang F, Gong J, Wang L, Wu Q, Li W, Liu H, Wang J, Zhang K, Li M, Huang X, Pu C, Li Y, Jiang F, Wang F, Min J, Chen X., Free PMC Article | 06/27/2020 |
We conclude that Hjv is required for inflammatory induction of hepcidin and controls the acute hypoferremic response | Hepcidin-mediated hypoferremic response to acute inflammation requires a threshold of Bmp6/Hjv/Smad signaling. Fillebeen C, Wilkinson N, Charlebois E, Katsarou A, Wagner J, Pantopoulos K. | 07/20/2019 |
Anti-hemojuvelin antibody corrects anemia caused by inappropriately high hepcidin levels | Anti-hemojuvelin antibody corrects anemia caused by inappropriately high hepcidin levels. Kovac S, Böser P, Cui Y, Ferring-Appel D, Casarrubea D, Huang L, Fung E, Popp A, Mueller BK, Hentze MW., Free PMC Article | 01/27/2018 |
These data suggest that, in Hjv(-/-) females, Bmp6 can provide a signal adequate to maintain hepcidin to a level sufficient to avoid extrahepatic iron loading. | Deletion of BMP6 worsens the phenotype of HJV-deficient mice and attenuates hepcidin levels reached after LPS challenge. Latour C, Besson-Fournier C, Gourbeyre O, Meynard D, Roth MP, Coppin H. | 12/16/2017 |
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 |
Hjv (--) and Hfe (C282YC282Y) transgenic mice displayed enhanced colonization of deep tissues by Yersinia pseudotuberculosis following oral inoculation, recapitulating enhanced susceptibility of humans with hemochromatosis to disseminated infection with enteropathogenic Yersinia. | Hereditary Hemochromatosis Predisposes Mice to Yersinia pseudotuberculosis Infection Even in the Absence of the Type III Secretion System. Miller HK, Schwiesow L, Au-Yeung W, Auerbuch V., Free PMC Article | 09/23/2017 |
The data demonstrate that endothelial cells are the predominant source of BMP6 in the liver and support a model in which endothelial cells BMP6 has paracrine actions on hepatocyte hemojuvelin to regulate hepcidin transcription and maintain systemic iron homeostasis. | Endothelial cells produce bone morphogenetic protein 6 required for iron homeostasis in mice. Canali S, Zumbrennen-Bullough KB, Core AB, Wang CY, Nairz M, Bouley R, Swirski FK, Babitt JL., Free PMC Article | 08/19/2017 |
The minor variant of the HJV polymorphic site rs16827043 is a significant factor associated with hypertension among 50 year-old individuals compared with the AA genotype carriers. For the other polymorphic variant rs7536827, association with hypertension was found only among normal or slightly overweight A-allele carriers. In conclusion, HJV genetic variants were associated with essential hypertension in Finnish subjects. | Minor variant of rs 16827043 in the iron regulator hemojuvelin gene (HJV) contributes to hypertension: The TAMRISK study. Nikkari ST, Visto AL, Määttä KM, Kunnas TA., Free PMC Article | 02/25/2017 |
Results indicate that an efficient induction of hepcidin expression by hemojuvelin (HJV) requires its interaction with neogenin. | Neogenin Facilitates the Induction of Hepcidin Expression by Hemojuvelin in the Liver. Zhao N, Maxson JE, Zhang RH, Wahedi M, Enns CA, Zhang AS., Free PMC Article | 12/17/2016 |
Single Hjv(-)/(-) and double Hfe(-)/(-)Hjv(-)/(-) mice exhibit comparable iron overload. Hfe and Hjv regulate hepcidin via the same pathway. | Hfe and Hjv exhibit overlapping functions for iron signaling to hepcidin. Kent P, Wilkinson N, Constante M, Fillebeen C, Gkouvatsos K, Wagner J, Buffler M, Becker C, Schümann K, Santos MM, Pantopoulos K. | 01/16/2016 |
Results show that HFE may depend on HJV for hepcidin regulation. Residual hepcidin in the absence of HFE suggests either the presence of an unknown regulator synergistic with HJV or that HJV is sufficient to maintain basal levels of hepcidin. | HJV and HFE Play Distinct Roles in Regulating Hepcidin. Wu Q, Wang H, An P, Tao Y, Deng J, Zhang Z, Shen Y, Chen C, Min J, Wang F., Free PMC Article | 01/16/2016 |
Parenchymal hepatic iron overload does not suffice to trigger progression of liver steatosis to steatohepatitis or fibrosis in Hjv knockout C57BL/6 mice. | A high-fat diet modulates iron metabolism but does not promote liver fibrosis in hemochromatotic Hjv⁻/⁻ mice. Padda RS, Gkouvatsos K, Guido M, Mui J, Vali H, Pantopoulos K. | 04/11/2015 |
Hjv is not required for sensing of body iron levels and merely functions as an enhancer for iron signaling to hepcidin. | Iron-dependent regulation of hepcidin in Hjv-/- mice: evidence that hemojuvelin is dispensable for sensing body iron levels. Gkouvatsos K, Fillebeen C, Daba A, Wagner J, Sebastiani G, Pantopoulos K., Free PMC Article | 09/20/2014 |
Deletion of Hjv in mice leads to abnormal retinal angiogenesis/vasculogenesis, with proliferation of new, leaky blood vessels in the vitreous. | Deletion of hemojuvelin, an iron-regulatory protein, in mice results in abnormal angiogenesis and vasculogenesis in retina along with reactive gliosis. Tawfik A, Gnana-Prakasam JP, Smith SB, Ganapathy V., Free PMC Article | 08/9/2014 |
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 |
Use of proteomic analysis enables identification of four disulfide linkages in hemojuvelin/repulsive guidance molecule C. This molecule is a single-chain HJV/RGMc isoform. | Proteomic analysis and molecular modelling characterize the iron-regulatory protein haemojuvelin/repulsive guidance molecule c. Nili M, David L, Elferich J, Shinde U, Rotwein P., Free PMC Article | 06/29/2013 |
we conclude that TNF-alpha suppresses Hemojuvelin(HJV) transcription possibly via a novel TNFRE within the HJV promoter | Tumour necrosis factor alpha downregulates human hemojuvelin expression via a novel response element within its promoter. Salama MF, Bayele HK, Srai SS., Free PMC Article | 04/6/2013 |
high levels of serum s-HJV in CDA I patients, suggesting that it may contribute to iron loading pathology in CDA I and eventually in other anemias with ineffective erythropoiesis. | High levels of soluble serum hemojuvelin in patients with congenital dyserythropoietic anemia type I. Shalev H, Perez-Avraham G, Kapelushnik J, Levi I, Rabinovich A, Swinkels DW, Brasse-Lagnel C, Tamary H. | 02/9/2013 |
Hemojuvelin is essential for transferrin-dependent and transferrin-independent hepcidin expression in conditions of iron overload. | Hemojuvelin is essential for transferrin-dependent and transferrin-independent hepcidin expression in mice. Bartnikas TB, Fleming MD., Free PMC Article | 09/22/2012 |
membrane-associated HJV is necessary for BMP6-mediated activation of hepcidin promoter inretinal pigment epithelium (RPE) cells; results confirm the biological importance of HJV in the regulation of iron homoeostasis in the retina and in RPE | Iron-mediated retinal degeneration in haemojuvelin-knockout mice. Gnana-Prakasam JP, Tawfik A, Romej M, Ananth S, Martin PM, Smith SB, Ganapathy V., Free PMC Article | 02/18/2012 |