ライフサイエンスコーパス: hemoglobin S

1 , persistent inhibition of polymerization of hemoglobin S.

2 e often milder in patients with heterozygous hemoglobin S.

3 ated conditions due to the polymerization of hemoglobin S.

4 hese ES cells produced both hemoglobin A and hemoglobin S.

5 ta-globin gene that causes polymerization of hemoglobin S.

6 ole for BP1 in determining the production of hemoglobin S.

7 hemoglobin A/S, and mixtures and hybrids of hemoglobin S.

8 produced under photolytic deliganding of CO hemoglobin S.

9 ote advantages, such as that associated with hemoglobin S.

10 s prodromal to most forms of brain injury in hemoglobin SS.

11 y, 300 adult patients with SCA were studied (hemoglobin SS = 184; and 116 with other sickling hemoglo

12 kle cell genotypes included 27 patients with hemoglobin SS (58.7%), 14 SC (30.4%), 4 beta-thalassemia

13 oglycerate, a potent allosteric inhibitor of hemoglobin's affinity for oxygen.

14 l linking the thermodynamics and kinetics of hemoglobin's allosteric (R --> T) and ligand binding rea

15 target the mutant beta(S)-globin subunits of hemoglobin-S (alpha(2)beta(S)(2)) for substitution by no

16 All subjects were assayed for hemoglobin S and hemoglobin C genotypes.

17 In this article, we show that hemoglobin S and normal adult hemoglobin, hemoglobin A,

18 that have erythrocytes containing only human hemoglobin S and that exhibit a degree of hemolytic anem

19 a process dependent on the concentration of hemoglobin S and the rate of deoxygenation, among other

20 HSCT for all children with SCD, particularly hemoglobin SS and Sbeta(0)-thalassemia disease, is ethic

21 he cerebral vasculature of mice that express hemoglobin-S (beta(s) mice).

22 in SC, hemoglobin S/beta(0)-thalassemia, and hemoglobin S/beta(+)-thalassemia).

23 or heterozygous hemoglobin S (hemoglobin SC, hemoglobin S/beta(0)-thalassemia, and hemoglobin S/beta(

24 model of SCD, that the adverse properties of hemoglobin-S can be reversed by exchanging its normal al

25 membrane nonheme iron levels were higher in hemoglobin SS cells than hemoglobin AA cells (0.0016 x 1

26 Cell-free hemoglobin's (CFH) high affinity for nitric oxide (NO) c

27 f the domain formation rate to intracellular hemoglobin S concentration explains the variable cell mo

28 tient received exchange transfusion, and his hemoglobin S concentration gradually decreased.

29 standard care or transfusions to reduce the hemoglobin S concentration to less than 30 percent of th

30 children were included; mean pretransfusion hemoglobin S concentration was 29%.

31 ization and sickling because of their higher hemoglobin S concentration.

32 cells from patients with sickle cell anemia, hemoglobin S denatures and forms Heinz bodies.

33 ated children with SCD homozygous for sickle hemoglobin (SS disease) and controls (n = 65) and demons

34 n) with sickle cell disease (4 patients with hemoglobin SS disease and 1 patient with hemoglobin SC d

35 In hemoglobin SS disease, increased AER (micro- and macroal

36 s, venules and capillaries and a decrease in hemoglobin's effectiveness for tissue oxygenation when i

37 mposed of 291 African American children with hemoglobin SS enrolled in the Cooperative Study for Sick

38 hat cytosolic labile iron is not elevated in hemoglobin SS erythrocytes and that elemental membrane i

39 This new technique, combining isolation of hemoglobin S fibers and measurement of micromechanical p

40 distribution of the birefringent domains of hemoglobin S fibers.

41 f the fully liganded fluoromet derivative of hemoglobin S (FmetHb S) were investigated by electron mi

42 setting, EBT is important to maintain a low hemoglobin S fraction peri- and posttransplantation.

43 inking, may apply to macroscopic rheology of hemoglobin S gels.

44 a, IL-10, MSP142 3D7 IgG antibody responses, hemoglobin S genotype, age, and infection status at base

45 nuclear cells from 13 subjects with SCD with hemoglobin SS genotype and 15 subjects with Chuvash poly

46 low in 21 patients with sickle cell disease (hemoglobin SS genotype) and 18 black control subjects be

47 Hemoglobin C (Glu beta 6-->Lys) shares with hemoglobin S (Glu beta 6-->Val) the site of mutation, bu

48 Common red blood cell polymorphisms (ie, hemoglobin S, glucose-6-phosphate dehydrogenase, and alp

49 Polymerization of a 1:1 mixture of hemoglobin S (Hb S) and the artificial mutant HbAbeta73L

50 The polymorphisms hemoglobin C (HbC) and hemoglobin S (HbS) - known to protect carriers from seve

51 Although hemoglobin S (HbS) and hemoglobin C (HbC) are well known

52 Hemoglobin S (HbS) and hemoglobin C (HbC) mutations are

53 therapy, the clinician lowers the amount of hemoglobin S (HbS) containing red blood cells (RBCs) by

54 cephalopathy and cholestatic jaundice with a Hemoglobin S (HbS) level of 69.6%.

55 Although hemoglobin S (HbS) polymerization and vaso-occlusion are

56 The mutant hemoglobin S (HbS) previously was reported to undergo ac

57 sickle cell disease is the polymerization of hemoglobin S (HbS) to form fibers that make red cells le

58 tate of normal hemoglobin A (HbA) and sickle hemoglobin S (HbS), confers protection against malaria i

59 The solubility of deoxygenated hemoglobin S (HbS), which is the concentration of fully

60 ich cells containing the mutated hemoglobin (hemoglobin S; HbS) will cause occlusion if they sickle i

61 ith homozygous hemoglobin SS or heterozygous hemoglobin S (hemoglobin SC, hemoglobin S/beta(0)-thalas

62 homozygous hemoglobin A (AA) or heterozygous hemoglobin S/hemoglobin A (SA) donor erythroid precursor

63 ildren who had tortuosity than percentage of hemoglobin S in children who had normal blood vessels at

64 sting implications for the polymerization of hemoglobin S in the sickle red cell.

65 in a cohort of 483 subjects with homozygous hemoglobin S in the U.S. and U.K. Walk-Treatment of Pulm

66 Findings suggest that greater percentage of hemoglobin S is associated with mild vasculopathy.

67 To describe hemoglobin's kinetics at the microscopic level of struct

68 disease, deoxygenation of intra-erythrocytic hemoglobin S leads to hemoglobin polymerization, erythro

69 Serial hemoglobin S levels were monitored, and measures were ta

70 nitored, and measures were taken to maintain hemoglobin S <20% to prevent sickle cell crisis.

71 While adults heterozygous for hemoglobin S mutation were less often parasitemic compar

72 total erythrocyte labile iron was similar in hemoglobin SS (n = 12) and hemoglobin AA (n = 10) partic

73 Substitution of this residue, precluding hemoglobin S-nitrosation, did not change total red blood

74 S-Nitrosation of cysteine beta93 in hemoglobin (S-nitrosohemoglobin (SNO-Hb)) occurs in vivo

75 opathy can occur in patients with homozygous hemoglobin SS or heterozygous hemoglobin S (hemoglobin S

76 Children 4 to 21 years old with hemoglobin SS or Sbeta(0) thalassemia requiring hospital

77 or a clinically relevant modification by the hemoglobin S phenotype of the effects of iron supplement

78 reases fetal hemoglobin (HbF), which reduces hemoglobin S polymerization and clinical complications.

79 Clinical manifestations resulting from hemoglobin S polymerization are often milder in patients

80 derstand the similarities and differences in hemoglobin S polymerization in the model compared to pol

81 Because hemoglobin S polymerization is profoundly influenced by

82 nowledge of how to properly apply studies of hemoglobin S polymerization that are conducted using the

83 Vaso-occlusion occurs when deoxygenated hemoglobin S polymerizes and erythrocytes sickle and adh

84 red cell membrane permeability generated by hemoglobin S polymers in the deoxygenated state.

85 nyl adduct to monitor religation kinetics to hemoglobin S polymers.

86 A cell was constructed in order to study hemoglobin's reaction with gaseous ligands.

87 n these disorders, polymerization of mutated hemoglobin S results in deformation of red blood cells,

88 Ineffective erythropoiesis of homozygous hemoglobin S (SS) progenitors thus provides a maturation

89 sed primarily by adherence of homozygous for hemoglobin S (SS) red blood cells (SSRBCs) and leukocyte

90 esidues alter the diffusion processes within hemoglobin's subunits and suggest that multiple pathways

91 ulopathy was more prevalent in patients with hemoglobin SS than in those with hemoglobin SC (P <.001)

92 l microscopy in mice expressing human sickle hemoglobin (SS) that SS red blood cells (RBCs) bind to a

93 se is caused by a mutant form of hemoglobin, hemoglobin S, that polymerizes under hypoxic conditions.

94 analysis for factor V Leiden, hemoglobin C, hemoglobin S, the thermolabile mutation of methylenetetr

95 resonance angiography (MRA) in children with hemoglobin SS, the most serious form of sickle cell dise

96 Erythrocytapheresis lowered the content of hemoglobin S to 5.6%, and therapeutic hypothermia was su

97 Hemoglobin C trait, like hemoglobin S trait, protects against severe malaria in c

98 ntially binds to the minor population of the hemoglobin's vacant hemes in a cooperative manner, nitro

99 A, hybrid formation between hemoglobin A and hemoglobin S was prevented, thus simplifying the analysi

100 ildren with sickle cell trait, percentage of hemoglobin S was significantly greater in children who h

101 d patients with hemolytic anemias not due to hemoglobin S were 2.6+/-1.6, 3.0+/-2.6, and 2.0+/-0.8 pe

102 omerular involvement, 76 adult SSA patients (hemoglobin SS) were studied to determine the relationshi

103 Dilution of hemoglobin S within erythrocytes, by stimulating fetal h