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Your search keyword '"Rivella, Stefano"' showing total 392 results
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392 results on '"Rivella, Stefano"'

1. TMPRSS6 as a Therapeutic Target for Disorders of Erythropoiesis and Iron Homeostasis.

Author

Ganz, Tomas, Nemeth, Elizabeta, Rivella, Stefano, Goldberg, Paul, Dibble, Andrew, McCaleb, Michael, Guo, Shuling, Monia, Brett, and Barrett, Terrance

Subjects
Hemochromatosis, Ineffective erythropoiesis, Iron overload, Iron restriction, Thalassemia, Mice, Animals, Humans, Erythropoiesis, Anemia, Iron-Deficiency, Iron, Liver, Homeostasis, Membrane Proteins, Serine Endopeptidases
Abstract

TMPRSS6 is a serine protease highly expressed in the liver. Its role in iron regulation was first reported in 2008 when mutations in TMPRSS6 were shown to be the cause of iron-refractory iron deficiency anemia (IRIDA) in humans and in mouse models. TMPRSS6 functions as a negative regulator of the expression of the systemic iron-regulatory hormone hepcidin. Over the last decade and a half, growing understanding of TMPRSS6 biology and mechanism of action has enabled development of new therapeutic approaches for patients with diseases of erythropoiesis and iron homeostasis.ClinicalTrials.gov identifier NCT03165864.

Published
2023

2. Use of HSC-targeted LNP to generate a mouse model of lethal α-thalassemia and treatment via lentiviral gene therapy

Author

Chappell, Maxwell E., Breda, Laura, Tricoli, Lucas, Guerra, Amaliris, Jarocha, Danuta, Castruccio Castracani, Carlo, Papp, Tyler E., Tanaka, Naoto, Hamilton, Nolan, Triebwasser, Michael P., Ghiaccio, Valentina, Fedorky, Megan T., Gollomp, Kandace L., Bochenek, Veronica, Roche, Aoife M., Everett, John K., Cook, Emma J., Bushman, Frederic D., Teawtrakul, Nattiya, Glentis, Stavros, Kattamis, Antonis, Mui, Barbara L., Tam, Ying K., Weissman, Drew, Abdulmalik, Osheiza, Parhiz, Hamideh, and Rivella, Stefano

Published
2024
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10. Pleckstrin-2 is essential for erythropoiesis in β-thalassemic mice, reducing apoptosis and enhancing enucleation

Author

Feola, Maria, Zamperone, Andrea, Moskop, Daniel, Chen, Huiyong, Casu, Carla, Lama, Dechen, Di Martino, Julie, Djedaini, Mansour, Papa, Luena, Martinez, Marc Ruiz, Choesang, Tenzin, Bravo-Cordero, Jose Javier, MacKay, Matthew, Zumbo, Paul, Brinkman, Nathan, Abrams, Charles S., Rivella, Stefano, Hattangadi, Shilpa, Mason, Christopher E., Hoffman, Ronald, Ji, Peng, Follenzi, Antonia, and Ginzburg, Yelena Z.

Published
2021
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14. Identification of erythroferrone as an erythroid regulator of iron metabolism

Author

Kautz, Léon, Jung, Grace, Valore, Erika V, Rivella, Stefano, Nemeth, Elizabeta, and Ganz, Tomas

Subjects
Cooley's Anemia, Hematology, Rare Diseases, Liver Disease, Digestive Diseases, Blood, Anemia, Animals, Blotting, Western, Disease Models, Animal, Enzyme-Linked Immunosorbent Assay, Epoetin Alfa, Erythropoiesis, Erythropoietin, Gene Expression Profiling, Hemoglobins, Hemorrhage, Hepcidins, Hormones, Iron, Iron Overload, Liver, Male, Mice, Mice, Inbred C57BL, Mice, Knockout, Molecular Sequence Data, RNA, Messenger, Real-Time Polymerase Chain Reaction, Recombinant Proteins, Reverse Transcriptase Polymerase Chain Reaction, beta-Thalassemia, Biological Sciences, Medical and Health Sciences, Developmental Biology
Abstract

Recovery from blood loss requires a greatly enhanced supply of iron to support expanded erythropoiesis. After hemorrhage, suppression of the iron-regulatory hormone hepcidin allows increased iron absorption and mobilization from stores. We identified a new hormone, erythroferrone (ERFE), that mediates hepcidin suppression during stress erythropoiesis. ERFE is produced by erythroblasts in response to erythropoietin. ERFE-deficient mice fail to suppress hepcidin rapidly after hemorrhage and exhibit a delay in recovery from blood loss. ERFE expression is greatly increased in Hbb(th3/+) mice with thalassemia intermedia, where it contributes to the suppression of hepcidin and the systemic iron overload characteristic of this disease.

Published
2014

28. In vivo hematopoietic stem cell modification by mRNA delivery.

Author

Breda, Laura, Papp, Tyler E., Triebwasser, Michael P., Yadegari, Amir, Fedorky, Megan T., Naoto Tanaka, Abdulmalik, Osheiza, Pavani, Giulia, Yongping Wang, Grupp, Stephan A., Chou, Stella T., Houping Ni, Mui, Barbara L., Tam, Ying K., Weissman, Drew, Rivella, Stefano, and Parhiz, Hamideh

Published
2023
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32. HMGB1 Mediates Anemia of Inflammation in Murine Sepsis Survivors

Author

Valdés-Ferrer, Sergio I., Papoin, Julien, Dancho, Meghan E., Olofsson, Peder S., Li, Jianhua, Lipton, Jeffrey M., Avancena, Patricia, Yang, Huan, Zou, Yong-Rui, Chavan, Sangeeta S., Volpe, Bruce T., Gardenghi, Sara, Rivella, Stefano, Diamond, Betty, Andersson, Ulf, Steinberg, Bettie M., Blanc, Lionel, and Tracey, Kevin J.

Published
2015
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34. Hepcidin is regulated by promoter-associated histone acetylation and HDAC3

Author

Pasricha, Sant-Rayn, Lim, Pei Jin, Duarte, Tiago L., Casu, Carla, Oosterhuis, Dorenda, Mleczko-Sanecka, Katarzyna, Suciu, Maria, Da Silva, Ana Rita, Al-Hourani, Kinda, Arezes, João, McHugh, Kirsty, Gooding, Sarah, Frost, Joe N., Wray, Katherine, Santos, Ana, Porto, Graça, Repapi, Emmanouela, Gray, Nicki, Draper, Simon J., Ashley, Neil, Soilleux, Elizabeth, Olinga, Peter, Muckenthaler, Martina U., Hughes, Jim R., Rivella, Stefano, Milne, Thomas A., Armitage, Andrew E., and Drakesmith, Hal

Published
2017
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35. Unexpected Expression of α- and β-globin in Mesencephalic Dopaminergic Neurons and Glial Cells

Author

Biagioli, Marta, Pinto, Milena, Cesselli, Daniela, Zaninello, Marta, Lazarevic, Dejan, Roncaglia, Paola, Simone, Roberto, Vlachouli, Christina, Plessy, Charles, Bertin, Nicolas, Beltrami, Antonio, Kobayashi, Kazuto, Gallo, Vittorio, Santoro, Claudio, Ferrer, Isidro, Rivella, Stefano, Beltrami, Carlo Alberto, Carninci, Piero, Raviola, Elio, Gustincich, Stefano, and Bizzi, Emilio

Published
2009
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41. Isocitrate ameliorates anemia by suppressing the erythroid iron restriction response

Author

Richardson, Chante L., Delehanty, Lorrie L., Bullock, Grant C., Rival, Claudia M., Tung, Kenneth S., Kimpel, Donald L., Gardenghi, Sara, Rivella, Stefano, and Goldfarb, Adam N.

Subjects
Anemia -- Research, Erythropoietin -- Research, Health care industry
Abstract

The unique sensitivity of early red cell progenitors to iron deprivation, known as the erythroid iron restriction response, serves as a basis for human anemias globally. This response impairs erythropoietin-driven [...]

Published
2013
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42. Reducing TMPRSS6 ameliorates hemochromatosis and β-thalassemia in mice

Author

Guo, Shuling, Casu, Carla, Gardenghi, Sara, Booten, Sheri, Aghajan, Mariam, Peralta, Raechel, Watt, Andy, Freier, Sue, Monia, Brett P., and Rivella, Stefano

Subjects
Antisense drugs -- Research, Proteases -- Health aspects, Hemochromatosis -- Genetic aspects -- Care and treatment, Thalassemia -- Genetic aspects -- Care and treatment, Health care industry
Abstract

β-Thalassemia and HFE-related hemochromatosis are 2 of the most frequently inherited disorders world-wide. Both disorders are characterized by low levels of hepcidin (HAMP), the hormone that regulates iron absorption. As [...]

Published
2013
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43. Macrophages support pathological erythropoiesis in polycythemia vera and β-thalassemia

Author

Ramos, Pedro, Casu, Carla, Gardenghi, Sara, Breda, Laura, Crielaard, Bart J., Guy, Ella, Marongiu, Maria Franca, Gupta, Ritama, Levine, Ross L., Abdel-Wahab, Omar, Ebert, Benjamin L., Rooijen, Nico Van, Ghaffari, Saghi, Grady, Robert W., Giardina, Patricia J., and Rivella, Stefano

Subjects
Macrophages -- Analysis -- Physiological aspects -- Research, Erythropoiesis -- Research -- Analysis -- Physiological aspects, Biological sciences, Health
Abstract

Regulation of erythropoiesis is achieved by the integration of distinct signals. Among them, macrophages are emerging as erythropoietin-complementary regulators of erythroid development, particularly under stress conditions. We investigated the contribution of macrophages to physiological and pathological conditions of enhanced erythropoiesis. We used mouse models of induced anemia, polycythemia vera and β-thalassemia in which macrophages were chemically depleted. Our data indicate that macrophages contribute decisively to recovery from induced anemia, as well as the pathological progression of polycythemia vera and b-thalassemia, by modulating erythroid proliferation and differentiation. We validated these observations in primary human cultures, showing a direct impact of macrophages on the proliferation and enucleation of erythroblasts from healthy individuals and patients with polycythemia vera or β-thalassemia. The contribution of macrophages to stress and pathological erythropoiesis, which we have termed stress erythropoiesis macrophage-supporting activity, may have therapeutic implications., Erythropoiesis is a dynamic and complex process during which eryth-roid progenitors develop into enucleated red blood cells (RBCs). This multistep program (1) is tightly regulated to allow for constant replenishment [...]

Published
2013
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45. Decreased differentiation of erythroid cells exacerbates ineffective erythropoiesis in β-thalassemia

Author

Libani, Ilaria V., Guy, Ella C., Melchiori, Luca, Schiro, Raffaella, Ramos, Pedro, Breda, Laura, Scholzen, Thomas, Chadburn, Amy, Liu, YiFang, Kernbach, Margrit, Baron-Lühr, Bettina, Porotto, Matteo, de Sousa, Maria, Rachmilewitz, Eliezer A., Hood, John D., Cappellini, M. Domenica, Giardina, Patricia J., Grady, Robert W., Gerdes, Johannes, and Rivella, Stefano

Published
2008
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48. Hepcidin as a therapeutic tool to limit iron overload and improve anemia in β-thalassemic mice

Author

Gardenghi, Sara, Ramos, Pedro, Marongiu, Maria Franca, Melchiori, Luca, Breda, Laura, Guy, Ella, Muirhead, Kristen, Rao, Niva, Roy, Cindy N., Andrews, Nancy C., Nemeth, Elizabeta, Follenzi, Antonia, An, Xiuli, Mohandas, Narla, Ginzburg, Yelena, Rachmilewitz, Eliezer A., Giardina, Patricia J., Grady, Robert W., and Rivella, Stefano

Subjects
Iron in the body -- Properties, Peptide hormones -- Dosage and administration, Anemia -- Drug therapy, Thalassemia -- Drug therapy, Health care industry
Abstract

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 liver, spleen, and kidneys markedly increases. Iron overload is associated with low levels of hepcidin, a peptide that regulates iron metabolism by triggering degradation of ferroportin, an iron-transport protein localized on absorptive enterocytes as well as hepatocytes and macrophages. Patients with β-thalassemia also have low hepcidin levels. These observations led us to hypothesize that more iron is absorbed in β-thalassemia than is required for erythropoiesis and that increasing the concentration of hepcidin in the body of such patients might be therapeutic, limiting iron overload. Here we demonstrate that a moderate increase in expression of hepcidin in β-thalassemic mice limits iron overload, decreases formation of insoluble membrane-bound globins and reactive oxygen species, and improves anemia. Mice with increased hepcidin expression also demonstrated an increase in the lifespan of their red cells, reversal of ineffective erythropoiesis and splenomegaly, and an increase in total hemoglobin levels. These data led us to suggest that therapeutics that could increase hepcidin levels or act as hepcidin agonists might help treat the abnormal iron absorption in individuals with β-thalassemia and related disorders., Introduction β-thalassemia is one of the most common congenital anemias arising from partial or complete lack of β-globin synthesis. β-thalassemia major, also known as Cooley anemia, is the most severe [...]

Published
2010
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50. Unexpected expression of [alpha]- and [beta]-globin in mesencephalic dopaminergic neurons and glial cells

Author

Biagioli, Marta, Pinto, Milena, Cesselli, Daniela, Zaninello, Marta, Lazarevic, Dejan, Roncaglia, Paola, Simone, Roberto, Vlachouli, Christina, Plessy, Charles, Bertin, Nicolas, Beltrami, Antonio, Kobayashi, Kazuto, Gallo, Vittorio, Santoro, Claudio, Ferrer, Isidro, Rivella, Stefano, Beltrami, Carlo Alberto, Carninci, Piero, Raviola, Elio, and Gustincich, Stefano

Subjects
Astrocytes -- Properties, Hemoglobin -- Properties, Oxidative phosphorylation -- Observations, Parkinson's disease -- Development and progression, Science and technology
Abstract

The mesencephalic dopaminergic (mDA) cell system is composed of two major groups of projecting cells in the substantia nigra (SN) (A9 neurons) and the ventral tegmental area (VTA) (A10 cells). A9 neurons form the nigrostriatal pathway and are involved in regulating voluntary movements and postural reflexes. Their selective degeneration leads to Parkinson's disease. Here, we report that gene expression analysis of A9 dopaminergic neurons (DA) identifies transcripts for [alpha]- and [beta]-chains of hemoglobin (Hb). Globin immunoreactivity decorates the majority of A9 DA, a subpopulati0n of cortical and hippocampal astrocytes and mature oligodendrocytes. This pattern of expression was confirmed in different mouse strains and in rat and human, We show that Hb is expressed in the SN of human postmortem brain. By microarray analysis of dopaminergic cell lines overexpressing [alpha]- and [beta]-globin chains, changes in genes involved in O2 homeostasis and oxidative phopshorylation were observed, linking Hb expression to mitochondrial function. Our data suggest that the most famed oxygen-carrying globin is not exclusively restricted to the blood, but it may play a role in the normal physiology of the brain and neurodegenerative diseases. astrocytes | hemoglobin | oligodendrocytes | oxidative phosphorylation | Parkinson

Published
2009

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