ライフサイエンスコーパス: hemoglobin S

1 , persistent inhibition of polymerization of hemoglobin S.

2 ell trait subjects compared to those lacking hemoglobin S.

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

4 e often milder in patients with heterozygous hemoglobin S.

5 ated conditions due to the polymerization of hemoglobin S.

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

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

8 el of SCD, HbSS-BERK mice express only human hemoglobin S.

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

10 produced under photolytic deliganding of CO hemoglobin S.

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

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

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

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

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

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

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

18 In sickle cell trait, low hemoglobin S and elevated hemoglobin A were associated w

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

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

21 suggesting an epistatic relationship between hemoglobin S and PIEZO1 E756del.

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

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

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

25 Deleterious alleles like hemoglobin S are well-known to confer strong resistance

26 fetal hemoglobin has been replaced by adult hemoglobin S at about 1 y after birth.

27 rapies require RBC transfusions to lower the hemoglobin S before cellular therapy collections and inf

28 on mechanisms that exploit the propensity of hemoglobin's beta subunit to release heme likely represe

29 eriae acquiring heme passively released from hemoglobin's beta subunits.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

44 36.2 (12.3) years; 119 patients (62.6%) had hemoglobin SS disease and 46 (24.2%) had hemoglobin SC d

45 with hemoglobin SC disease, 14 (25.9%) with hemoglobin SS disease, 13 (24.1%) with sickle cell trait

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

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

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

49 e-cell trait, balancing selection results in hemoglobin S equilibrium allele frequencies of between 1

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

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

52 distribution of the birefringent domains of hemoglobin S fibers.

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

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

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

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

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

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

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

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

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

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

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

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

65 ease results from compound heterozygosity of hemoglobin S (HbS) and hemoglobin C (HbC), comprising 30

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

67 scence to detect the point mutation encoding hemoglobin S (HbS) in one round of isothermal recombinas

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

69 t beta (HBB) gene containing the site of the hemoglobin S (HbS) mutation as well as to a paralogous h

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

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

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

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

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

75 high oxygen affinity R (oxy) conformation of hemoglobin S (HbS).

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

77 children aged 1 to 10 years with homozygous hemoglobin S (HbSS) in a randomized, open-label trial co

78 duce VOE in the Townes sickle homozygous for hemoglobin S (HbSS) mouse model and complement inhibitor

79 es, dyslipidemias, and obesity, resulting in hemoglobin SS (HbSS), hemoglobin SC (HbSC), and SCT coho

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

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

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

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

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

85 adults (aged 40.1 +/- 13.7 y, 83 women, 87% hemoglobin SS) in a stable state enrolled consecutively

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

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

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

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

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

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

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

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

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

95 ms in four major red cell genes that lead to hemoglobin S, O blood group, alpha-thalassemia, and the

96 nfounders of malaria risk, including age and hemoglobin S or C, were similar between individuals with

97 ticipants had sickle cell anemia (homozygous hemoglobin S or hemoglobin Sbeta(0)-thalassemia), and ap

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

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

100 ally higher percentage of female, older, and hemoglobin SS participants compared to the ACS group.

101 883]; 95% CI, 2.828 to 10.296; P < .001) and hemoglobin S percentage (beta [SE] per 1% increase, 0.08

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

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

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

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

106 Because hemoglobin S polymerization is profoundly influenced by

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

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

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

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

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

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

113 ies and 60 cities; 388 individuals with SCD (hemoglobin SS, SC, S-B0 thalassemia, or S-B+ thalassemia

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

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

116 nal thinning than those with the sickle cell hemoglobin SS subtype.

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

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

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

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

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

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

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

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

125 Polymerization of deoxygenated hemoglobin S underlies the pathophysiology of sickle cel

126 is caused by polymerization of the abnormal hemoglobin S upon deoxygenation in the tissues to form f

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

128 Low hemoglobin S was associated with faster eGFR decline in

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

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

131 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

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

133 ith SCD-VOE (13.6 +/- 3 years; 67% male; 75% hemoglobin-SS) were randomized to 1 of 3 arginine doses:

134 ted blood disorder marked by homozygosity of hemoglobin S, which is a defective hemoglobin caused by

135 , and the compound heterozygous condition of hemoglobin S with pancellular hereditary persistence of

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