Zobrazeno 1 - 10
of 95
pro vyhledávání: '"Robert W, Grady"'
Autor:
Robert W. Grady, Renzo Galanello, Rachel E. Randolph, Dorothy A. Kleinert, Carlo Dessi, Patricia J. Giardina
Publikováno v:
Haematologica, Vol 98, Iss 1 (2013)
Patients with β-thalassemia require iron chelation therapy to protect against progressive iron overload and non-transferrin-bound iron. Some patients fail to respond adequately to deferoxamine and deferasirox monotherapy while others have side effec
Externí odkaz:
https://doaj.org/article/b74d058b039c474ba9fc9bedc9e56537
Autor:
Laura Breda, Carla Casu, Sara Gardenghi, Nicoletta Bianchi, Luca Cartegni, Mohandas Narla, Karina Yazdanbakhsh, Marco Musso, Deepa Manwani, Jane Little, Lawrence B Gardner, Dorothy A Kleinert, Eugenia Prus, Eitan Fibach, Robert W Grady, Patricia J Giardina, Roberto Gambari, Stefano Rivella
Publikováno v:
PLoS ONE, Vol 7, Iss 3, p e32345 (2012)
Preclinical and clinical studies demonstrate the feasibility of treating β-thalassemia and Sickle Cell Disease (SCD) by lentiviral-mediated transfer of the human β-globin gene. However, previous studies have not addressed whether the ability of len
Externí odkaz:
https://doaj.org/article/472254d68557495b9619e90809e22289
Autor:
Pedro Ramos, Nico van Rooijen, Bart J Crielaard, Benjamin L. Ebert, Omar Abdel-Wahab, Sara Gardenghi, Saghi Ghaffari, Robert W. Grady, Carla Casu, Ritama Gupta, Ross L. Levine, Ella Guy, Laura Breda, Stefano Rivella, Maria F. Marongiu, Patricia J. Giardina
Publikováno v:
Nature medicine
Ramos, P, Casu, C, Gardenghi, S, Breda, L, Crielaard, B J, Guy, E, Marongiu, M F, Gupta, R, Levine, R L, Abdel-Wahab, O, Ebert, B L, van Rooijen, N, Ghaffari, S, Grady, R W, Giardina, P J & Rivella, S 2013, ' Macrophages support pathological erythropoiesis in polycythemia vera and beta-thalassemia ', Nature Medicine, vol. 19, no. 4, pp. 437-445 . https://doi.org/10.1038/nm.3126
Nature Medicine, 19(4), 437-445. Nature Publishing Group
Ramos, P, Casu, C, Gardenghi, S, Breda, L, Crielaard, B J, Guy, E, Marongiu, M F, Gupta, R, Levine, R L, Abdel-Wahab, O, Ebert, B L, van Rooijen, N, Ghaffari, S, Grady, R W, Giardina, P J & Rivella, S 2013, ' Macrophages support pathological erythropoiesis in polycythemia vera and beta-thalassemia ', Nature Medicine, vol. 19, no. 4, pp. 437-445 . https://doi.org/10.1038/nm.3126
Nature Medicine, 19(4), 437-445. Nature Publishing Group
Regulation of erythropoiesis is achieved by integration of distinct signals. Among these, macrophages are emerging as erythropoietin-complementary regulators of erythroid development, particularly under stress conditions. We investigated the contribu
Autor:
Janet L. Kwiatkowski, Robert W. Grady, Daniele Alberti, Elliott Vichinsky, Annie Higa, Patricia J. Giardina, Patricia Evans, John B. Porter, Paul Harmatz, Felicia Trachtenberg, Thomas D. Coates, Bruce N. Ames, Patrick B. Walter, Ellen B. Fung, Ellis J. Neufeld, Nancy F. Olivieri
Publikováno v:
British Journal of Haematology. 160:399-403
This exploratory study assessed apoptosis in peripheral blood leucocytes (PBL) from β-thalassaemia patients receiving chronic transfusions and chelation therapy (deferasirox or deferoxamine) at baseline, 1, 6, and 12 months. At baseline, thalassaemi
Autor:
Renzo Galanello, Carlo Dessì, Robert W. Grady, Rachel Randolph, Patricia J. Giardina, Dorothy A. Kleinert
Publikováno v:
Haematologica. 98:129-135
Patients with β-thalassemia require iron chelation therapy to protect against progressive iron overload and non-transferrin-bound iron. Some patients fail to respond adequately to deferoxamine and deferasirox monotherapy while others have side effec
Autor:
Antonia Follenzi, Robert W. Grady, Sara Gardenghi, Nico van Rooijen, Ella Guy, Stefano Rivella, Pedro Ramos, Catia C. Proenca, Carla Casu, Nan Chen, Maria de Sousa
Publikováno v:
Blood, 117(4), 1379-1389. American Society of Hematology
Ramos, P, Guy, E, Chen, N, Proenca, C C, Gardenghi, S, Casu, C, Follenzi, A, van Rooijen, N, Grady, R W, de Sousa, M & Rivella, S 2011, ' Enhanced erythropoiesis in Hfe-KO mice indicates a role for Hfe in the modulation of erythroid iron homeostasis ', Blood, vol. 117, no. 4, pp. 1379-1389 . https://doi.org/10.1182/blood-2010-09-307462
Ramos, P, Guy, E, Chen, N, Proenca, C C, Gardenghi, S, Casu, C, Follenzi, A, van Rooijen, N, Grady, R W, de Sousa, M & Rivella, S 2011, ' Enhanced erythropoiesis in Hfe-KO mice indicates a role for Hfe in the modulation of erythroid iron homeostasis ', Blood, vol. 117, no. 4, pp. 1379-1389 . https://doi.org/10.1182/blood-2010-09-307462
In hereditary hemochromatosis, mutations in HFE lead to iron overload through abnormally low levels of hepcidin. In addition, HFE potentially modulates cellular iron uptake by interacting with transferrin receptor, a crucial protein during erythropoi
Autor:
Antonia Follenzi, Ella Guy, Xiuli An, Laura Breda, Elizabeta Nemeth, Cindy N. Roy, Nancy C. Andrews, Patricia J. Giardina, Niva Rao, Maria F. Marongiu, Pedro Ramos, Yelena Ginzburg, Luca Melchiori, Eliezer A. Rachmilewitz, Stefano Rivella, Sara Gardenghi, Kristen Muirhead, Robert W. Grady, Narla Mohandas
Publikováno v:
Journal of Clinical Investigation. 120:4466-4477
Excessive iron absorption is one of the main features of β-thalassemia and can lead to severe morbidity and mortality. Serial analyses of β-thalassemic mice indicate that while hemoglobin levels decrease over time, the concentration of iron in the
Publikováno v:
Hematology/Oncology Clinics of North America. 24:1089-1107
β-Thalassemia is a genetic disorder caused by mutations in the β-globin gene and characterized by chronic anemia caused by ineffective erythropoiesis, and accompanied by a variety of serious secondary complications such as extramedullary hematopoie
Autor:
Renzo Galanello, Fabrice Danjou, Robert W. Grady, Simona Campus, Patricia J. Giardina, Annalisa Agus
Publikováno v:
Annals of the New York Academy of Sciences. 1202:79-86
Patients with thalassemia major accumulate body iron over time as a consequence of continuous red blood cell transfusions which cause hepatic, endocrine, and cardiac complications. Despite the availability of three iron chelators, some patients fail
Autor:
Pedro Ramos, Eliezer A. Rachmilewitz, Antonia Follenzi, Sara Gardenghi, Niva Rao, Robert W. Grady, Patricia J. Giardina, Stefano Rivella
Publikováno v:
Annals of the New York Academy of Sciences. 1202:221-225
Hepcidin (HAMP) negatively regulates iron absorption, degrading the iron exporter ferroportin at the level of enterocytes and macrophages. We showed that mice with β-thalassemia intermedia (th3/+) have increased anemia and iron overload. However, th