Academic literature on the topic 'Erythropoietin receptor, Thalassemia, Hemoglobin, Parkinson's disease'

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Journal articles on the topic "Erythropoietin receptor, Thalassemia, Hemoglobin, Parkinson's disease"

1

Galanello, R., S. Barella, MP Turco, et al. "Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin beta-thalassemia intermedia patients." Blood 83, no. 2 (1994): 561–65. http://dx.doi.org/10.1182/blood.v83.2.561.561.

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Abstract Clinical data suggest that in beta-thalassemia-intermedia patients, higher levels of circulating fetal hemoglobin (HbF) are associated with greater disease severity at comparable degrees of anemia. We assessed the influence of the amount of circulating HbF on serum erythropoietin (s-Epo) levels and on serum transferrin receptor, a measure of erythropoiesis, in 30 beta-thalassemia-intermedia patients. Twenty-four showed more than 40% HbF (21 of whom with beta (0)-thalassemia) and 6 presented lower HbF levels (beta(+)-thalassemia). The two groups of patients did not differ in age (15.3
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Galanello, R., S. Barella, MP Turco, et al. "Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin beta-thalassemia intermedia patients." Blood 83, no. 2 (1994): 561–65. http://dx.doi.org/10.1182/blood.v83.2.561.bloodjournal832561.

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Clinical data suggest that in beta-thalassemia-intermedia patients, higher levels of circulating fetal hemoglobin (HbF) are associated with greater disease severity at comparable degrees of anemia. We assessed the influence of the amount of circulating HbF on serum erythropoietin (s-Epo) levels and on serum transferrin receptor, a measure of erythropoiesis, in 30 beta-thalassemia-intermedia patients. Twenty-four showed more than 40% HbF (21 of whom with beta (0)-thalassemia) and 6 presented lower HbF levels (beta(+)-thalassemia). The two groups of patients did not differ in age (15.3 v 19 year
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Fibach, Eitan, and Johnny Amer. "The In Vitro and In Vivo Antioxidant Effects of Erythropoietin in Thalassemia." Blood 110, no. 11 (2007): 573. http://dx.doi.org/10.1182/blood.v110.11.573.573.

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Abstract Erythropoietin (EPO), a hormone produced on hypoxia mainly in the kidneys, enhances red blood cell (RBC) production (erythropoiesis) by stimulating the proliferation of erythroid progenitors and precursors in the bone marrow. This effect is mediated by the homodimeric EPO receptor, a class 1 cytokine receptor. Recombinant human EPO is widely used for the treatment of anemia, e.g., in patients on dialysis, patients with myelodysplastic syndrome and oncology patients undergoing chemotherapy. Treatment with EPO was also tried experimentally in patients with thalassemia. In these patients
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Mahagna, Lila, Osama Tanous, Tal Dujovny та ін. "Leptin Is Associated with the Degree of Anemia and the Erythropoietin Levels in β Thalassemia Patients". Blood 130, Suppl_1 (2017): 950. http://dx.doi.org/10.1182/blood.v130.suppl_1.950.950.

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Abstract Background: β-thalassemia (BT) is a hereditary hemolytic anemia. The imbalance between α- and β-globin chain synthesis results in ineffective erythropoiesis, severe microcytic hypochromic anemia and iron overload. Although regular transfusions and iron-chelation therapy markedly improve the survival and quality of life of BT patients, they have also led to the emergence of previously unrecognized complications. Patients with thalassemia major often present with endocrine abnormalities due to dysfunction of the hypothalamic-pituitary axis (Poggi, 2016), and are frequently underweight w
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Lob, Heinrich E., Leah Kravets, Lawrence Miloscio, Jason Mastaitis, Aris N. Economides та Sarah J. Hatsell. "Anti-GDF11Treatment of β-Thalassemia Intermedia Mice Does Not Improve Erythropoiesis". Blood 136, Supplement 1 (2020): 7–8. http://dx.doi.org/10.1182/blood-2020-140605.

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Beta-thalassemia is a hereditary iron-independent anemia, caused by a reduction of β-globin, affecting millions of people globally. Current treatments such as blood transfusions and iron chelation show significant toxicities and add to organ damage. Luspatercept (Acvr2b(L79D)-Fc) is a novel treatment for transfusion-dependent β-thalassemia patients that improves erythropoiesis independent of erythropoietin. The exact mechanism of action remains unknown, and it is controversially discussed if growth differentiation factor 11 (GDF11) is the main target of this drug. Genetic models raised doubts
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Chu, Simon N., Eric Soupene, Beeke Wienert та ін. "Dual α-Globin and Truncated EPO Receptor Knockin Restores Hemoglobin Production in α-Thalassemia-Derived Hematopoietic Stem and Progenitor Cells". Blood 142, Supplement 1 (2023): 485. http://dx.doi.org/10.1182/blood-2023-180187.

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Introduction: α-thalassemia major (ATM) is an autosomal recessive disorder where all four copies of the α-globin gene are deleted. A traditionally lethal disease due to severe fetal hypoxia, this condition is now survivable due to the advent of in utero blood transfusions. However, patients still require life-long transfusions postnatally. While allogeneic hematopoietic stem cell transplant (HSCT) can provide a definitive cure, it is limited by the paucity of suitable donors, has a high morbidity and mortality due the prerequisite myeloablative regimens, and carries the risk of immune rejectio
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Cazzola, M., L. Ponchio, F. de Benedetti, et al. "Defective iron supply for erythropoiesis and adequate endogenous erythropoietin production in the anemia associated with systemic-onset juvenile chronic arthritis." Blood 87, no. 11 (1996): 4824–30. http://dx.doi.org/10.1182/blood.v87.11.4824.bloodjournal87114824.

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Systemic-onset juvenile chronic arthritis (SoJCA) is associated with high levels of circulating interleukin-6 (IL-6) and is frequently complicated by severe microcytic anemia whose pathogenesis is unclear. Therefore, we studied 20 consecutive SoJCA patients with hemoglobin (Hb) levels <12 g/dL, evaluating erythroid progenitor proliferation, endogenous erythropoietin production, body iron status, and iron supply for erythropoiesis. Hb concentrations ranged from 6.5 to 11.9 g/dL. Hb level was directly related to mean corpuscular volume (r = .82, P < .001) and inversely related to circulati
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Casu, Carla, Pedro Ramos, Luca Melchiori, et al. "Potential Therapeutic Applications of Jak2 Inhibitors in Beta-Thalassemia and Sickle Cell Disease,." Blood 118, no. 21 (2011): 3187. http://dx.doi.org/10.1182/blood.v118.21.3187.3187.

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Abstract Abstract 3187 ß-Thalassemia and sickle cell disease (SCD) are the most common genetic red blood cell (RBC) disorders characterized respectively by limited production of aberrant ß-globin chains. In both cases, chronic transfusions and iron chelation are required to treat the anemia and/or formation of abnormal RBC. In ß-thalassemia, anemia stimulates erythropoietin (Epo) synthesis, which in turn leads to increased erythropoiesis and development of hepatosplenomegaly, often resulting in the need for splenectomy. Recently, we demonstrated that erythroid cells from ß-thalassemic mice hav
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Guerrero, Manuel Ugidos, Sujit Sheth, Olivier Hermine та Sadanand Vodala. "Improvement of Underlying Disease Pathophysiology of Ineffective Erythropoiesis in Non-Transfusion-Dependent (NTD) Patients with β-Thalassemia Receiving Luspatercept: Biomarker Analysis from the BEYOND Trial". Blood 142, Supplement 1 (2023): 1104. http://dx.doi.org/10.1182/blood-2023-179122.

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Background: Ineffective erythropoiesis and chronic hemolytic anemia are hallmarks of β-thalassemia which ultimately lead to extramedullary hematopoiesis (EMH), iron overload and other complications. Patients with hemoglobin levels chronically < 10 g/dL are at higher risk for clinical complications and mortality. A significant proportion of patients treated with luspatercept on the BEYOND study (NCT03342404) achieved ≥ 1.0 g/dL hemoglobin increases from baseline, with a manageable safety profile. This increase of hemoglobin is associated with improvement in health-related quality of life
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Di Modica, Simona Maria, Emanuele Tanzi, Alessia Pagani, Laura Silvestri та Antonella Nai. "Hyperactive Erythropoiesis Induced By Hematopoietic Tfr2 Deletion Corrects Glucose Abnormalities in β-Thalassemic Mice". Blood 144, Supplement 1 (2024): 3852. https://doi.org/10.1182/blood-2024-207290.

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Introduction: β-thalassemia is a genetic disease characterized by anemia, ineffective erythropoiesis and iron-overload due to inadequate hemoglobin production, which leads to premature cell death. Alterations of glucose metabolism and diabetes are common complications of the disease, usually ascribed to organ dysfunction because of iron accumulation. However, β-thalassemia carriers, who do not present iron-overload, have a higher risk of developing diabetes compared to the general population, suggesting that factors other than iron may contribute to glycometabolic abnormalities. Transferrin Re
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Dissertations / Theses on the topic "Erythropoietin receptor, Thalassemia, Hemoglobin, Parkinson's disease"

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Marcuzzi, Federica. "Erythropoietin Receptor and Hemoglobin, two commonly blood – associated proteins, in mesencephalic dopaminergic neurons." Doctoral thesis, SISSA, 2012. http://hdl.handle.net/20.500.11767/4720.

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Dopaminergic neurons (DA) are an anatomically and functionally heterogeneous group of cells involved in a wide range of neuronal network activities and behaviour. Among them, mesencephalic dopaminergic neurons (mDA) are the major source of dopamine in the brain. They present two main groups of projecting cells: the A9 neurons of the Substantia Nigra (SN) and the A10 cells of the Ventral Tegmental Area (VTA). A9 neurons form the nigrostriatal pathway and are involved in regulating voluntary movements and postural reflexes. Their selective degeneration leads to Parkinson’s disease (PD) and the l
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