ライフサイエンスコーパス: thalassemic

1 a pathogenetic feature shared by parasitized thalassemic and G6PD-deficient RBCs, triggering abnormal

2 in the spleen, bone marrow, and liver of the thalassemic animals.

3 As a result, the thalassemic beta-globin pre-mRNAs are spliced almost exc

4 For beta-thalassemic CD34(+) cells, similar gene transfer efficie

5 Thalassemic cells were associated with expression of cel

6 In isolated thalassemic cells, short-term proteasome inhibition bloc

7 This is the first reported case of a thalassemic condition that is maintained in a species as

8 These mice mimic the human alpha-thalassemic conditions and can be used as preclinical mo

9 n this locus such as sickle cell disease and thalassemic diseases.

10 icant overexpression of beta-globin in alpha-thalassemic disorders.

11 ate from the blood cells of one of four beta-thalassemic donors and 3H-alpha-chains or 3H-alpha-globi

12 ng effect on somatic recombination events in thalassemic embryos.

13 s, with heme attached to them, accumulate in thalassemic erythroblasts causing oxidative stress and t

14 In beta-thalassemic erythrocyte precursors, free alpha-globin wa

15 rovide a framework for studying the enhanced thalassemic erythroid apoptosis.

16 vel of total endogenous alpha-globin mRNA in thalassemic erythroid cells resulted in improved red cel

17 ances translation of ATF4 mRNA in mouse beta-thalassemic erythroid precursors.

18 globin gene will be required to improve beta-thalassemic erythropoiesis?

19 larly effective in individuals carrying beta-thalassemic gene defects.

20 t proteolysis pathway in unfractionated beta-thalassemic hemolysates.

21 Mice transplanted with beta-thalassemic HSCs transduced with a gamma-globin/MGMT vec

22 Using BALB/c donors and beta-thalassemic HW-80 recipients, we found significantly imp

23 experiments performed on blood from HbE/beta thalassemics in the temperature range 39 degrees C to 41

24 s substitutes for deficient adult globins in thalassemic individuals.

25 ls of thalassemia, aberrant splicing of beta-thalassemic IVS2-705 pre-mRNA was permanently corrected

26 was increased in splenic erythroblasts from thalassemic mice and in erythroblasts and sera from subj

27 Generation of beta-thalassemic mice chimeric for a minority proportion of g

28 r deferiprone (L1) intraperitoneally to beta-thalassemic mice for 4 wk and then studied RBC survival

29 Erfe and the consequences of its ablation in thalassemic mice from 3 to 12 weeks of age.

30 These cells were transplanted into beta-thalassemic mice given nonmyeloablative pretransplantati

31 Serial analyses of beta-thalassemic mice indicate that while hemoglobin levels d

32 The loss of ERFE in thalassemic mice led to full restoration of hepcidin mRN

33 e increase in expression of hepcidin in beta-thalassemic mice limits iron overload, decreases formati

34 in utero transplantation in nonanemic, beta-thalassemic mice to study chimerism, tolerance, and chan

35 eparate set of independent experiments, beta-thalassemic mice were bred with transgenic mice that exp

36 Moreover, thalassemic mice with established iron overload had sign

37 Injection of thalassemic mice with SCF plus nanoparticles containing

38 ontrast, prolonged in vivo treatment of beta-thalassemic mice with the proteasome inhibitor bortezomi

39 as been shown to be highly expressed in beta-thalassemic mice, restores hepcidin levels and corrects

40 In thalassemic mice, we observed that a greater than normal

41 s confirmed by up-regulation of TfR2 in beta-thalassemic mice, which, like hypotransferrinemic mice,

42 sg1 expression were performed in healthy and thalassemic mice.

43 d a Jak2 inhibitor, TG101209, to healthy and thalassemic mice.

44 xpression by 52%, yielding 9 g Hb/VC in beta-thalassemic mice.

45 production and correction of anemia in beta-thalassemic mice.

46 Transfection of HeLa cells expressing the 3 thalassemic mutants with modified U7 snRNA (U7.623), con

47 HbCS) is the most common nondeletional alpha-thalassemic mutation and is an important cause of HbH-li

48 rable populations of hemoglobin E (HbE)/beta thalassemics, one regularly transfused and one receiving

49 learance of rigid pathological cells such as thalassemic or sickle cells, and even to interactions of

50 In this study, CD34+ cells from 31 thalassemic patients mobilized with hydroxyurea+granuloc

51 rythropoietic cells from transgenic mice and thalassemic patients suggests the applicability of this

52 Mononuclear cells from peripheral blood of thalassemic patients were treated with morpholino oligon

53 Seventy-nine thalassemic patients without a history of chelation ther

54 Our data indicate that in a number of thalassemic patients, subpopulations of red cells circul

55 the improvement of hemolytic anemia in beta-thalassemic patients.

56 Hamp modulation provides improvement of the thalassemic phenotype of the Hbb(th3/+) mouse.

57 s can result in severe and frequently lethal thalassemic phenotypes.

58 errant 5' or 3' splice sites in the IVS2-705 thalassemic pre-mRNA.

59 This premature destruction of the thalassemic RBC could in part be due to a loss of phosph

60 he presence of PS-exposing subpopulations of thalassemic RBC that are most likely physiologically imp

61 n deposits contribute to the pathobiology of thalassemic RBC.

62 Analysis by fluorescent microscopy of beta-thalassemic RBCs indicates that PS on the outer leaflet

63 The results indicate that in the context of thalassemic splicing mutations and possibly in other alt

64 of animals that received transplants of beta-thalassemic stem cells transduced with a new vector cont

65 Without iron-deficient or thalassemic subjects, the difference had narrowed to 6.1

66 lobin genes in mice results in corresponding thalassemic syndromes that are uniformly fatal in utero.

67 the molecular abnormalities that led to the thalassemic syndromes, it still is not known how accumul

68 zed membranes from HbCS and deletional alpha-thalassemic variants and found that in addition to oxidi

69 s more severe when compared with other alpha-thalassemic variants.