-
1
-
-
0026099699
-
Beyond hemoglobin polymerization: The red blood cell membrane and sickle disease pathophysiology
-
Hebbe RP: Beyond hemoglobin polymerization: the red blood cell membrane and sickle disease pathophysiology. Blood 1991, 77:214-237.
-
(1991)
Blood
, vol.77
, pp. 214-237
-
-
Hebbe, R.P.1
-
3
-
-
0029819804
-
A recombinant sickle hemoglobin triple mutant with independent inhibitory effects on polymerization
-
Himanen JP, Mirza UA, Chait BT, Bookchin RM, Manning Jm: A recombinant sickle hemoglobin triple mutant with independent inhibitory effects on polymerization. J Biol Chem 1996, 271:25152-25156.
-
(1996)
J Biol Chem
, vol.271
, pp. 25152-25156
-
-
Himanen, J.P.1
Mirza, U.A.2
Chait, B.T.3
Bookchin, R.M.4
Manning, Jm.5
-
4
-
-
0006936094
-
Structural bases of the inhibitory effects of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S
-
Nagel RL, Bookchin RM, Johnson J, Labie D, Wajcman H, Isaac-Sodeye WA, Honig GR, et al.: Structural bases of the inhibitory effects of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S. Proc Natl Acad Sci U S A 1979, 76:670-672.
-
(1979)
Proc Natl Acad Sci U S A
, vol.76
, pp. 670-672
-
-
Nagel, R.L.1
Bookchin, R.M.2
Johnson, J.3
Labie, D.4
Wajcman, H.5
Isaac-Sodeye, W.A.6
Honig, G.R.7
-
5
-
-
0025770390
-
Pain in sickle cell disease. Rates and risk factors
-
Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, Kinney TR: Pain in sickle cell disease. Rates and risk factors. N Engl J Med 1991, 325:11-16.
-
(1991)
N Engl J Med
, vol.325
, pp. 11-16
-
-
Platt, O.S.1
Thorington, B.D.2
Brambilla, D.J.3
Milner, P.F.4
Rosse, W.F.5
Vichinsky, E.6
Kinney, T.R.7
-
6
-
-
0029025475
-
Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia
-
Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SVX, McMahon RP, Bonds DR: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995, 332:1317-1322.
-
(1995)
N Engl J Med
, vol.332
, pp. 1317-1322
-
-
Charache, S.1
Terrin, M.L.2
Moore, R.D.3
Dover, G.J.4
Barton, F.B.5
Eckert, S.V.X.6
McMahon, R.P.7
Bonds, D.R.8
-
7
-
-
0029982591
-
Hydroxyurea therapy in children severely affected with sickle cell disease
-
Scott JP, Hillery CA, Brown ER, Misiewicz V, Labotka RJ: Hydroxyurea therapy in children severely affected with sickle cell disease. J Pediatr 1996, 128:820-828.
-
(1996)
J Pediatr
, vol.128
, pp. 820-828
-
-
Scott, J.P.1
Hillery, C.A.2
Brown, E.R.3
Misiewicz, V.4
Labotka, R.J.5
-
8
-
-
0029845949
-
Hydroxyurea for treatment of severe sickle cell anemia: A pediatric clinical trial
-
Ferster A, Vermylen C, Cornu G, Buyse M, Corazza F, Deralck C, Fondu P, et al.: Hydroxyurea for treatment of severe sickle cell anemia: a pediatric clinical trial. Blood 1996, 88:1960-1964.
-
(1996)
Blood
, vol.88
, pp. 1960-1964
-
-
Ferster, A.1
Vermylen, C.2
Cornu, G.3
Buyse, M.4
Corazza, F.5
Deralck, C.6
Fondu, P.7
-
9
-
-
0030358260
-
Clinical and hematologic effects of hydroxyurea in children with sickle cell anemia
-
Jayabose S, Tugal O, Sandoval C, Patel P, Puder D, Lin T, Visintainer P: Clinical and hematologic effects of hydroxyurea in children with sickle cell anemia. J Pediatr 1996, 129:559-565.
-
(1996)
J Pediatr
, vol.129
, pp. 559-565
-
-
Jayabose, S.1
Tugal, O.2
Sandoval, C.3
Patel, P.4
Puder, D.5
Lin, T.6
Visintainer, P.7
-
10
-
-
0029795425
-
First report of reversal of organ dysfunction in sickle cell anemia by the use of hydroxyurea: Splenic regeneration
-
Claster S, Vichinsky E: First report of reversal of organ dysfunction in sickle cell anemia by the use of hydroxyurea: splenic regeneration. Blood 1996, 88:1951-1953.
-
(1996)
Blood
, vol.88
, pp. 1951-1953
-
-
Claster, S.1
Vichinsky, E.2
-
11
-
-
0030464953
-
Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive "switching" agent. the Multicenter Study of Hydroxyurea in Sickle Cell Anemia
-
Charache S, Barton FB, Moore RD, Terrin ML, Steinberg MH, Dover GJ, Ballas SK, et al.: Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive "switching" agent. The Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Medicine 1996, 75:300-326.
-
(1996)
Medicine
, vol.75
, pp. 300-326
-
-
Charache, S.1
Barton, F.B.2
Moore, R.D.3
Terrin, M.L.4
Steinberg, M.H.5
Dover, G.J.6
Ballas, S.K.7
-
12
-
-
10544232620
-
A multiparameter analysis of sickle erythrocytes in patients undergoing hydroxyurea therapy
-
Bridges KR, Barabino GD, Brugnara C, Cho MR, Christoph GW, Dover G, Ewenstein BM, et al.: A multiparameter analysis of sickle erythrocytes in patients undergoing hydroxyurea therapy. Blood 1996, 88:4701-4710.
-
(1996)
Blood
, vol.88
, pp. 4701-4710
-
-
Bridges, K.R.1
Barabino, G.D.2
Brugnara, C.3
Cho, M.R.4
Christoph, G.W.5
Dover, G.6
Ewenstein, B.M.7
-
13
-
-
0030895689
-
Decrease of very late activation antlgen-4 and CD36 on reticulocytes in sickle cell patients treated with hydroxyurea
-
Styles LA, Lubin B, Vichinsky E, Lawrence S, Hua M, et al.: Decrease of very late activation antlgen-4 and CD36 on reticulocytes in sickle cell patients treated with hydroxyurea. Blood 1997, 89:2554-2559.
-
(1997)
Blood
, vol.89
, pp. 2554-2559
-
-
Styles, L.A.1
Lubin, B.2
Vichinsky, E.3
Lawrence, S.4
Hua, M.5
-
14
-
-
0027078611
-
A short-term trial of butyrate to stimulate fetal-globin-gene expression in the beta-globin disorders
-
Perrine SP, Ginder GD, Faller DV, Dover GS, Ikuta T, Witkowska HE, Cai SP, et al.: A short-term trial of butyrate to stimulate fetal-globin-gene expression in the beta-globin disorders. N Engl J Med 1993, 328:81-86.
-
(1993)
N Engl J Med
, vol.328
, pp. 81-86
-
-
Perrine, S.P.1
Ginder, G.D.2
Faller, D.V.3
Dover, G.S.4
Ikuta, T.5
Witkowska, H.E.6
Cai, S.P.7
-
15
-
-
0029032965
-
Extended therapy with intravenous arginine butyrate in patients with beta-hemoglobinopathies
-
Sher GD, Ginder GD, Little J, Yang S, Dover GJ, Olivieri NF, et al.: Extended therapy with intravenous arginine butyrate in patients with beta-hemoglobinopathies. N Engl J Med 1995, 332:1606-1610.
-
(1995)
N Engl J Med
, vol.332
, pp. 1606-1610
-
-
Sher, G.D.1
Ginder, G.D.2
Little, J.3
Yang, S.4
Dover, G.J.5
Olivieri, N.F.6
-
16
-
-
0029681871
-
Effects of density and of dehydration of sickle cells on their adhesion to cultured endothelial cells
-
Stone PC, Stuart J, Nash GB: Effects of density and of dehydration of sickle cells on their adhesion to cultured endothelial cells. Am J Hematol 1996, 52:135-143.
-
(1996)
Am J Hematol
, vol.52
, pp. 135-143
-
-
Stone, P.C.1
Stuart, J.2
Nash, G.B.3
-
17
-
-
0024539761
-
Autoxidation, dehydration, and adhesivity may be related abnormalities of sickle erythrocytes
-
Hebbel RP, Ney PA, Foker W: Autoxidation, dehydration, and adhesivity may be related abnormalities of sickle erythrocytes. Am J Physiol 1989, 256:C579-C583.
-
(1989)
Am J Physiol
, vol.256
-
-
Hebbel, R.P.1
Ney, P.A.2
Foker, W.3
-
18
-
-
0022549289
-
Regulation of erythrocyte cation and water content in sickle cell anemia
-
Brugnara C, Bunn HF, Tosteson DC: Regulation of erythrocyte cation and water content in sickle cell anemia. Science 1986, 232:388-390.
-
(1986)
Science
, vol.232
, pp. 388-390
-
-
Brugnara, C.1
Bunn, H.F.2
Tosteson, D.C.3
-
19
-
-
0029966766
-
Dehydration of transferrin receptor-positive sickle reticulocytes during continuous or cyclic deoxygenation: Role of KCI cotransport and extracellular calcium
-
Franco RS, Palascak M, Thompson H, Rucknagel OL, Joiner CH, et al.: Dehydration of transferrin receptor-positive sickle reticulocytes during continuous or cyclic deoxygenation: role of KCI cotransport and extracellular calcium. Blood 1996, 88:4359-4365.
-
(1996)
Blood
, vol.88
, pp. 4359-4365
-
-
Franco, R.S.1
Palascak, M.2
Thompson, H.3
Rucknagel, O.L.4
Joiner, C.H.5
-
20
-
-
0029666477
-
+ channel in human erythrocytes and alters cell volume and filterability
-
+ channel in human erythrocytes and alters cell volume and filterability. J Biol Chem 1996, 271:18651-18656.
-
(1996)
J Biol Chem
, vol.271
, pp. 18651-18656
-
-
Li, Q.1
Jungmann, V.2
Kiyatkin, A.3
Low, P.S.4
-
22
-
-
0019119824
-
A study of induced hyponatremia in the prevention and treatment of sickle-cell crisis
-
Rosa RM, Bierer BE, Thomas R, Staff JS, Kruskall M, Robinson S, Stoff JS, Kruskall M, Robinson S, et al.: A study of induced hyponatremia in the prevention and treatment of sickle-cell crisis. N Engl J Med 1980, 303:1138-1143.
-
(1980)
N Engl J Med
, vol.303
, pp. 1138-1143
-
-
Rosa, R.M.1
Bierer, B.E.2
Thomas, R.3
Staff, J.S.4
Kruskall, M.5
Robinson, S.6
Stoff, J.S.7
Kruskall, M.8
Robinson, S.9
-
23
-
-
0029793542
-
Modulation of erythrocyte potassium chloride cotransport, potassium content, and density by dietary magnesium intake in transgenic SAD mouse
-
De Franceschi L, Beuzard Y, Jouault H, Brugnara C: Modulation of erythrocyte potassium chloride cotransport, potassium content, and density by dietary magnesium intake in transgenic SAD mouse. Blood 1996, 88:2738-2744. Treatment of SAD sickle mice (a transgenic mouse model that partially mimics sickle cell disease) with oral magnesium resulted decreased activity of the K/CI cotransport system and improved hydration status of the mouse erythrocytes. Reduced hemolysis was also evident, as shown by an increase in the hemoglobin and a decrease in the reticulocyte count.
-
(1996)
Blood
, vol.88
, pp. 2738-2744
-
-
De Franceschi, L.1
Beuzard, Y.2
Jouault, H.3
Brugnara, C.4
-
25
-
-
0029865706
-
Therapy with oral clotrimazole induces inhibition of the Gardos channel and reduction of erythrocyte dehydration in patients with sickle cell disease
-
+ (Gardos) channel, decreased erythrocyte dehydration, and a striking reduction of the number of dense red cells. A mild reduction in hemolysis with an increase in hemoglobin and a decrease in bilirubin also was evident in the majority of clotrimazole-treated patients.
-
(1996)
J Clin Invest
, vol.97
, pp. 1227-1234
-
-
Brugnara, C.1
Gee, B.2
Armsby, C.C.3
Kurth, S.4
Sakamoto, M.5
-
26
-
-
0018924116
-
Abnormal adherence of sickle erythrocytes to cultured vascular endothelium: Possible mechanism for microvascular occlusion in sickle cell disease
-
Hebbel RP, Yamada O, Moldow CF, Jacob HS, White JG, Eaton JW, Jacob HS, White JG, Eaton JW: Abnormal adherence of sickle erythrocytes to cultured vascular endothelium: possible mechanism for microvascular occlusion in sickle cell disease. J Clin Invest 1980, 65:154-160.
-
(1980)
J Clin Invest
, vol.65
, pp. 154-160
-
-
Hebbel, R.P.1
Yamada, O.2
Moldow, C.F.3
Jacob, H.S.4
White, J.G.5
Eaton, J.W.6
Jacob, H.S.7
White, J.G.8
Eaton, J.W.9
-
27
-
-
0022405050
-
Sickle erythrocyte adherence to vascular endothelium. Morphologic correlates and the requirement for divalent cations and collagen-binding plasma proteins
-
Mohandas N, Evans E: Sickle erythrocyte adherence to vascular endothelium. Morphologic correlates and the requirement for divalent cations and collagen-binding plasma proteins. J Clin Invest 1985, 76:1605-1612.
-
(1985)
J Clin Invest
, vol.76
, pp. 1605-1612
-
-
Mohandas, N.1
Evans, E.2
-
28
-
-
0023186247
-
Endothelial cell interactions with sickle cell, sickle trait, mechanically injured, and normal erythrocytes under controlled flow
-
Barabino GA, McIntire LV, Eskin SG, Sears DA, Udden M: Endothelial cell interactions with sickle cell, sickle trait, mechanically injured, and normal erythrocytes under controlled flow. Blood 1987, 70:152-157.
-
(1987)
Blood
, vol.70
, pp. 152-157
-
-
Barabino, G.A.1
McIntire, L.V.2
Eskin, S.G.3
Sears, D.A.4
Udden, M.5
-
29
-
-
0024395606
-
Modeling sickle cell vasoocclusion in the rat leg: Quantification of trapped sickle cells and correlation with 31P metabolic and 1H magnetic resonance imaging changes
-
Fabry ME, Rajanayagam V, Fine E, Holl S and Gore JC, et al.: Modeling sickle cell vasoocclusion in the rat leg: quantification of trapped sickle cells and correlation with 31P metabolic and 1H magnetic resonance imaging changes. Proc Natl Acad Sci U S A 1989, 86:3808-3812.
-
(1989)
Proc Natl Acad Sci U S A
, vol.86
, pp. 3808-3812
-
-
Fabry, M.E.1
Rajanayagam, V.2
Fine, E.3
Holl, S.4
Gore, J.C.5
-
30
-
-
0344812230
-
Microvascular sites and characteristics of sickle cell adhesion to vascular endothelium in shear flow conditions: Pathophysiological implications
-
Kaul DK, Fabry ME, Nagel RL: Microvascular sites and characteristics of sickle cell adhesion to vascular endothelium in shear flow conditions: pathophysiological implications. Proc Natl Acad Sci U S A 1989, 86:3356-3360.
-
(1989)
Proc Natl Acad Sci U S A
, vol.86
, pp. 3356-3360
-
-
Kaul, D.K.1
Fabry, M.E.2
Nagel, R.L.3
-
31
-
-
0030912619
-
Mechanisms of stroke in sickle cell disease: Role of the nitric oxide pathway in a rat cerebral microvascular model
-
French JA II, Kenny D, Scott JP, Hoffman RG, Wood JD, Hudetz AG, Hillery CA: Mechanisms of stroke in sickle cell disease: role of the nitric oxide pathway In a rat cerebral microvascular model. Blood 1997, 89:4591-4599. Washed human red cells were infused into rats prepared with either a cranial window or laser Doppler flow probes to monitor cerebral microvascular blood flow. When human sickle red cells were infused into rats pretreated with a nitric oxide synthase inhibitor, the majority of treated rats rapidly developed stroke and subsequently died. This suggests that the nitric oxide pathway is critical in maintaining organ blood flow in the presence of sickle red cells in this rat model.
-
(1997)
Blood
, vol.89
, pp. 4591-4599
-
-
French II, J.A.1
Kenny, D.2
Scott, J.P.3
Hoffman, R.G.4
Wood, J.D.5
Hudetz, A.G.6
Hillery, C.A.7
-
32
-
-
0028881822
-
In vivo demonstration of red cell-endothelial interaction, sickling and altered microvascular response to oxygen in the sickle transgenic mouse
-
Kaul DK, Fabry ME, Costantini F, Rubin EM, Nagel RL: In vivo demonstration of red cell-endothelial interaction, sickling and altered microvascular response to oxygen in the sickle transgenic mouse. J Clin Invest 1995, 96:2845-2853.
-
(1995)
J Clin Invest
, vol.96
, pp. 2845-2853
-
-
Kaul, D.K.1
Fabry, M.E.2
Costantini, F.3
Rubin, E.M.4
Nagel, R.L.5
-
33
-
-
0027184968
-
4β1-integrin expression on sickle reticulocytes: Vascular cell adhesion molecule-1-dependent binding to endothelium
-
4β1-integrin expression on sickle reticulocytes: vascular cell adhesion molecule-1-dependent binding to endothelium. Blood 1993, 82:1891-1899.
-
(1993)
Blood
, vol.82
, pp. 1891-1899
-
-
Swerlick, R.A.1
Eckman, J.R.2
Kumar, A.3
Jeitler, M.4
Wick, T.A.5
-
35
-
-
0026474688
-
Thrombospondin mediates adherence of CD36+ sickle reticulocytes to endothelial cells
-
Sugihara K, Sugihara T, Mohandas N, Hebbel RP: Thrombospondin mediates adherence of CD36+ sickle reticulocytes to endothelial cells. Blood 1992, 80:2634-2642.
-
(1992)
Blood
, vol.80
, pp. 2634-2642
-
-
Sugihara, K.1
Sugihara, T.2
Mohandas, N.3
Hebbel, R.P.4
-
36
-
-
0030119698
-
CD36-positive stress reticulocytosis in sickle cell
-
Browne PV, Hebbel RP: CD36-positive stress reticulocytosis in sickle cell. J Lab Clin Med 1996, 127:340-347.
-
(1996)
J Lab Clin Med
, vol.127
, pp. 340-347
-
-
Browne, P.V.1
Hebbel, R.P.2
-
37
-
-
0029897658
-
Increased adhesion of erythrocytes to components of the extracellular matrix: Isolation and characterization of a red blood cell lipid that binds thrombospondin and laminin
-
Hillery CA, Du MC, Montgomery RR, Scott JP: Increased adhesion of erythrocytes to components of the extracellular matrix: isolation and characterization of a red blood cell lipid that binds thrombospondin and laminin. Blood 1996, 87:4879-4886.
-
(1996)
Blood
, vol.87
, pp. 4879-4886
-
-
Hillery, C.A.1
Du, M.C.2
Montgomery, R.R.3
Scott, J.P.4
-
38
-
-
0028894111
-
Sickle reticulocytes adhere to VCAM-1 Sickle reticulocytes adhere to VCAM-1
-
Gee BE, Platt OS: Sickle reticulocytes adhere to VCAM-1 Sickle reticulocytes adhere to VCAM-1. Blood 1995, 85:268-274.
-
(1995)
Blood
, vol.85
, pp. 268-274
-
-
Gee, B.E.1
Platt, O.S.2
-
39
-
-
0030033081
-
Adhesion of sickle neutrophils and erythrocytes to fibronectin
-
Kasschau MR, Barabino GA, Bridges KR, Golan DE: Adhesion of sickle neutrophils and erythrocytes to fibronectin. Blood 1996, 87:771-780.
-
(1996)
Blood
, vol.87
, pp. 771-780
-
-
Kasschau, M.R.1
Barabino, G.A.2
Bridges, K.R.3
Golan, D.E.4
-
40
-
-
0027477197
-
Thrombospondin from activated platelets promotes sickle erythrocyte adherence to human microvascular endothelium under physiologic flow: A potential role for platelet activation in sickle cell vaso-occlusion
-
Brittain HA, Eckman JR, Swerlick RA, Howard RJ, Wick TM: Thrombospondin from activated platelets promotes sickle erythrocyte adherence to human microvascular endothelium under physiologic flow: a potential role for platelet activation in sickle cell vaso-occlusion. Blood 1993, 81:2137-2143.
-
(1993)
Blood
, vol.81
, pp. 2137-2143
-
-
Brittain, H.A.1
Eckman, J.R.2
Swerlick, R.A.3
Howard, R.J.4
Wick, T.M.5
-
41
-
-
0029062761
-
Platelet glycoprotein IIb/IIIa receptors in cardiovascular medicine
-
Lefkovits J, Plow EF, Topol EJ: Platelet glycoprotein IIb/IIIa receptors in cardiovascular medicine. N Engl J Med 1995, 332:1553-1559.
-
(1995)
N Engl J Med
, vol.332
, pp. 1553-1559
-
-
Lefkovits, J.1
Plow, E.F.2
Topol, E.J.3
-
42
-
-
0029973833
-
Adhesion of sickle red blood cells and damage to interieukin-1 beta stimulated endothelial cells under flow in vitro
-
Natarajan M, Udden MM, McIntire LV: Adhesion of sickle red blood cells and damage to interieukin-1 beta stimulated endothelial cells under flow in vitro. Blood 1996, 87:4845-4852.
-
(1996)
Blood
, vol.87
, pp. 4845-4852
-
-
Natarajan, M.1
Udden, M.M.2
McIntire, L.V.3
-
43
-
-
0029089199
-
Sickle erythrocytes, after sickling, regulate the expression of the endothelin-1 gene and protein in human endothelial cells in culture
-
Phelan M, Perrine SP, Brauer M, Faller DV: Sickle erythrocytes, after sickling, regulate the expression of the endothelin-1 gene and protein in human endothelial cells in culture. J Clin Invest 1995, 96:1145-1151.
-
(1995)
J Clin Invest
, vol.96
, pp. 1145-1151
-
-
Phelan, M.1
Perrine, S.P.2
Brauer, M.3
Faller, D.V.4
-
44
-
-
0029875840
-
S-nitrosohaemoglobin: A dynamic activity of blood involved in vascular control
-
Jia L, Bonaventura C, Bonaventura J, Stamler JS: S-nitrosohaemoglobin: a dynamic activity of blood involved in vascular control. Nature 1996, 380:221-226.
-
(1996)
Nature
, vol.380
, pp. 221-226
-
-
Jia, L.1
Bonaventura, C.2
Bonaventura, J.3
Stamler, J.S.4
-
45
-
-
0030982523
-
Low concentrations of nitric oxide increase oxygen affinity of sickle erythrocytes in vitro and in vivo
-
Head CA, Brugnara C, Martinez-Ruiz R, Kacmarek RM, Bridges KR, Kuter D, et al.: Low concentrations of nitric oxide increase oxygen affinity of sickle erythrocytes in vitro and in vivo. J Clin Invest 1997, 100:1193-1198. Nine asymptomatic patients with sickle cell disease and three healthy controls were treated with inhaled nitric oxide, with minimal methemoglobin production and no other significant toxicity noted. The sickle red cells, but not control red cells, developed increased oxygen affinity during nitric oxide therapy. The increased oxygen affinity of the nitric oxide-treated sickle red cells could be therapeutically beneficial in reducing deoxyhemoglobin S polymerization.
-
(1997)
J Clin Invest
, vol.100
, pp. 1193-1198
-
-
Head, C.A.1
Brugnara, C.2
Martinez-Ruiz, R.3
Kacmarek, R.M.4
Bridges, K.R.5
Kuter, D.6
-
46
-
-
9344231922
-
Bone marrow transplantation for sickle cell disease
-
Walters MC, Patience M, Leisenring W, Eckman JR, Scott JP, Mentzer WC, et al.: Bone marrow transplantation for sickle cell disease. N Engl J Med 1996, 335:369-376. Reports the results of a multicenter trial in which 22 patients with severe sickle cell disease received allogenic bone marrow transplantation. Twenty patients survived, and 16 patients had stable engraftment resulting in stabilization of endorgan damage and a clinical cure of the sickle cell disease.
-
(1996)
N Engl J Med
, vol.335
, pp. 369-376
-
-
Walters, M.C.1
Patience, M.2
Leisenring, W.3
Eckman, J.R.4
Scott, J.P.5
Mentzer, W.C.6
-
47
-
-
0029817766
-
Correction of the mutation responsible for sickle cell anemia by an RNA-DNA oligonucleotide
-
Cole-Strauss A, Yoon K, Xiang Y, et al.: Correction of the mutation responsible for sickle cell anemia by an RNA-DNA oligonucleotide. Science 1996, 273:1386-1389. A chimeric RNA-DNA oligonucleotide coding for normal beta hemoglobin was introduced into nonerythroid B cells homozygous for the hemoglobin S mutation and resulted in detectable correction of the hemoglobin S gene mutation. Although the success of this technique is currently cell line dependent, this may be a potential approach for the treatment of genetic disorders in the future.
-
(1996)
Science
, vol.273
, pp. 1386-1389
-
-
Cole-Strauss, A.1
Yoon, K.2
Xiang, Y.3
-
48
-
-
1842408336
-
Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease
-
Paszty C, Brion CM, Manci E, Wltkowska HE, Stevens ME, Mohandasn, Rubin EM, et al.: Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease. Science 1997, 278:876-878. A report of a transgenic sickle cell mouse line that expresses exclusively human sickle and fetal hemoglobin. These mice phentypically express many of the manifestations of human sickle cell disease including a severe hemolytic anemia, irreversibly sickled erythrocytes, multiorgan dysfunction, and a high perinatal mortality.
-
(1997)
Science
, vol.278
, pp. 876-878
-
-
Paszty, C.1
Brion, C.M.2
Manci, E.3
Wltkowska, H.E.4
Stevens, M.E.5
Mohandasn6
Rubin, E.M.7
-
49
-
-
0030725279
-
Knockout-transgenic mouse model of sickle cell disease
-
Ryan TM, Ciavatta DJ, Townes TM: Knockout-transgenic mouse model of sickle cell disease. Science 1997, 278:873-876. A report of a second transgenic sickle cell mouse line expressing human sickle and fetal hemoglobin. Although these mice similarly express many phenotypic manifestations of human sickle cell disease, a higher level of fetal hemoglobin is expressed at birth and a higher perinatal survival rate is observed. Therefore, both of these sickle cell mice lines are excellent models to study the pathophysiology of sickle cell disease and to test potential new therapies for this disorder.
-
(1997)
Science
, vol.278
, pp. 873-876
-
-
Ryan, T.M.1
Ciavatta, D.J.2
Townes, T.M.3
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