Search Results - "NAGEL, Ronald L"

Two-Step Mechanism of Homogeneous Nucleation of Sickle Cell Hemoglobin Polymers by Galkin, Oleg, Pan, Weichun, Filobelo, Luis, Hirsch, Rhoda Elison, Nagel, Ronald L., Vekilov, Peter G.

“…Sickle cell anemia is a debilitating genetic disease that affects hundreds of thousands of babies born each year worldwide. Its primary pathogenic event is the…”
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Heme degradation and oxidative stress in murine models for hemoglobinopathies: Thalassemia, sickle cell disease and hemoglobin C disease by Nagababu, Enika, Fabry, Mary E., Nagel, Ronald L., Rifkind, Joseph M.

“…Red blood cells with abnormal hemoglobins (Hb) are frequently associated with increased hemoglobin autoxidation, accumulation of iron in membranes, increased…”
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Liquid-Liquid Separation in Solutions of Normal and Sickle Cell Hemoglobin by Galkin, Oleg, Chen, Kai, Nagel, Ronald L., Hirsch, Rhoda Elison, Vekilov, Peter G.

“…We show that in solutions of human hemoglobin (Hb)-oxy- and deoxy-Hb A or S-of near-physiological pH, ionic strength, and Hb concentration, liquid-liquid phase…”
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Permanent and Panerythroid Correction of Murine β Thalassemia by Multiple Lentiviral Integration in Hematopoietic Stem Cells by Imren, Suzan, Payen, Emmanuel, Westerman, Karen A., Pawliuk, Robert, Fabry, Mary E., Eaves, Connie J., Cavilla, Benjamin, Wadsworth, Louis D., Beuzard, Yves, Bouhassira, Eric E., Russell, Robert, London, Irving M., Nagel, Ronald L., Leboulch, Philippe, Humphries, R. Keith

“…Achieving long-term pancellular expression of a transferred gene at therapeutic level in a given hematopoietic lineage remains an important goal of gene…”
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Sickle-cell disease by Stuart, Marie J, Nagel, Ronald L

“…With the global scope of sickle-cell disease, knowledge of the countless clinical presentations and treatment of this disorder need to be familiar to…”
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Liganded Hemoglobin Structural Perturbations by the Allosteric Effector L35 by Chen, Qiuying, Lalezari, Iraj, Nagel, Ronald L., Hirsch, Rhoda Elison

“…Effector binding to liganded hemoglobin (Hb) provides a new understanding of structural determinants of Hb function. L35, a bezafibrate-related compound, is…”
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Metastable Mesoscopic Clusters in Solutions of Sickle-Cell Hemoglobin by Pan, Weichun, Galkin, Oleg, Filobelo, Luis, Nagel, Ronald L., Vekilov, Peter G.

“…Sickle cell hemoglobin (HbS) is a mutant, whose polymerization while in deoxy state in the venous circulation underlies the debilitating sickle cell anemia. It…”
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Implications of the Genetic Epidemiology of Globin Haplotypes Linked to the Sickle Cell Gene in Southern Iran by Rahimi, Zohreh, Merat, Ahmad, Gerard, Nathalie, Krishnamoorthy, Rajagopal, Nagel, Ronald L.

“…To determine the origin of sickle cell mutation in different ethnic groups living in southern Iran, we studied the haplotype background of the βS and βA genes…”
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The Kinetics of Nucleation and Growth of Sickle Cell Hemoglobin Fibers by Galkin, Oleg, Nagel, Ronald L., Vekilov, Peter G.

“…Polymerization of sickle cell hemoglobin (HbS) in deoxy state is one of the basic events in the pathophysiology of sickle cell anemia. For insight into the…”
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Correction of Sickle Cell Disease in Transgenic Mouse Models by Gene Therapy by Pawliuk, Robert, Westerman, Karen A., Fabry, Mary E., Payen, Emmanuel, Tighe, Robert, Bouhassira, Eric E., Acharya, Seetharama A., Ellis, James, London, Irving M., Eaves, Connie J., Humphries, R. Keith, Beuzard, Yves, Nagel, Ronald L., Leboulch, Philippe

“…Sickle cell disease (SCD) is caused by a single point mutation in the human βAglobin gene that results in the formation of an abnormal hemoglobin [HbS (α2βS…”
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Epistasis and the genetics of human diseases by Nagel, Ronald L

“…Epistasis or modifier genes, that is, gene-gene interactions of non-allelic partners, play a major role in susceptibility to common human diseases. This old…”
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The paradox of hemoglobin SC disease by Nagel, Ronald L., Fabry, Mary E., Steinberg, Martin H.

“…Homozygous HbC gene results only in mild hemolytic anemia. In HbSC disease red cells contain equal levels of HbS and HbC. It is a paradox that HbSC exhibit a…”
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High-level beta-globin expression and preferred intragenic integration after lentiviral transduction of human cord blood stem cells by Imren, Suzan, Fabry, Mary E, Westerman, Karen A, Pawliuk, Robert, Tang, Patrick, Rosten, Patricia M, Nagel, Ronald L, Leboulch, Philippe, Eaves, Connie J, Humphries, R Keith

“…Transplantation of genetically corrected autologous hematopoietic stem cells is an attractive approach for the cure of sickle-cell disease and…”
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Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice by Kaul, Dhananjay K, Liu, Xiao-du, Chang, Hee-Yoon, Nagel, Ronald L, Fabry, Mary E

“…In sickle cell disease, intravascular sickling and attendant flow abnormalities underlie the chronic inflammation and vascular endothelial abnormalities…”
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Molecular characterization of glucose-6-phosphate dehydrogenase deficiency in the Kurdish population of Western Iran by Rahimi, Zohreh, Vaisi-Raygani, Asad, Nagel, Ronald L., Muniz, Adriana

“…A total of 1000 school boys ages 14–18 years, were randomly selected from six schools of Kermanshah and screened for G6PD deficiency. Fifty-three out of 1000…”
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Inhibition of sickle red cell adhesion and vasoocclusion in the microcirculation by antioxidants by Kaul, Dhananjay K, Liu, Xiao-du, Zhang, Xiaoqin, Ma, Li, Hsia, Carleton J C, Nagel, Ronald L

“…In sickle cell anemia (SCA), inflammatory (i.e., intravascular sickling and transient vasoocclusive) events result in chronic endothelial activation. In…”
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Prevalence of factor V Leiden (G1691A) and prothrombin (G20210A) among Kurdish population from Western Iran by Rahimi, Zohreh, Vaisi-Raygani, Asad, Mozafari, Hadi, Kharrazi, Hadi, Rezaei, Mansour, Nagel, Ronald L.

“…Background The mutation in factor V (FV) G1691A, known as factor V Leiden, and prothrombin (FII) gene G20210A are the two most prevalent causes of inherited…”
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Thrombophilic mutations among Southern Iranian patients with sickle cell disease: high prevalence of factor V Leiden by Rahimi, Zohreh, Vaisi-Raygani, Asad, Nagel, Ronald L., Muniz, Adriana

“…Background A hypercoagulable state in sickle cell disease (SCD) and beta thalassemia has been established and thrombosis is an important aspect of the clinical…”
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Prevalence of thrombotic risk factors among β-thalassemia patients from Western Iran by Rahimi, Zohreh, Ghaderi, Mandana, Nagel, Ronald L., Muniz, Adriana

“…Background There is evidence for increased risk of thrombosis in patients with β-thalassemia intermedia and β-thalassemia major. The present study investigated…”
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Exogenous iron increases hemoglobin in β–thalassemic mice by Ginzburg, Yelena Z, Rybicki, Anne C, Suzuka, Sandra M, Hall, Charles B, Breuer, William, Cabantchik, Z. Ioav, Bouhassira, Eric E, Fabry, Mary E, Nagel, Ronald L

“…Objective β–thalassemia results from β–globin gene mutations that lead to ineffective erythropoiesis, shortened red cell survival, and anemia. Patients with…”
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