ライフサイエンスコーパス: HHT

1 HHT [corrected] shows considerable variation in clinical

2 HHT and the transcription inhibitor SNS-032 induced syne

3 HHT at pH 6.6 resulted in denaturation of approximately

4 HHT inhibited protein synthesis and reduced the Bcr-Abl

5 HHT is a very effective treatment of early chronic phase

6 HHT is characterized by development of fragile, direct c

7 HHT manifests highly variable incidence and severity of

8 HHT patients presented a significantly lower rate of hea

9 HHT type 2 is caused by loss of function mutations in ac

10 HHT types 1 and 2 are caused by loss of function mutatio

11 HHT-associated missense mutations in the ALK-1 extracell

12 HHT/MDA synthase activity was present in purified P450s

13 and 12(S)-hydroxyheptadecatrienoic acid (12-HHT), protects mice from lung injury caused by a pneumoc

14 or loxoprofen inhibited the production of 12-HHT and increased the sensitivity toward PLY, which was

15 led randomized clinical trial performed at 6 HHT centers of excellence.

16 2C12 cells expressing BMP9-unresponsive ALK1 HHT mutants and in HHT patient blood outgrowth ECs.

17 ion in Acvrl1 were carefully examined for an HHT-like phenotype.

18 te that accelerated recovery of HHT-5371 and HHT-5411 decreased accumulation of these channels in ina

19 For mutants HHT-5411 and HHT-5371, the residual current appeared associated with

20 The combinations of flavopiridol and HHT and flavopiridol and imatinib synergistically decrea

21 were not cross-resistant to flavopiridol and HHT.

22 eriogenesis, and vascular disorders, such as HHT and pulmonary hypertension.

23 Because HHT is caused by loss-of-function mutations in bone morp

24 Furthermore, both HHT-5371 and -5411 recovered from inactivation significa

25 fludarabine, this induction was reversed by HHT, which overcame stromal cell-mediated protection.

26 ized that inhibition of protein synthesis by HHT would decrease Mcl-1 expression and induce apoptosis

27 ifferent genes, endoglin or ALK-1, can cause HHT.

28 n one of the additional genes that may cause HHT.

29 cular mechanism by which ENG mutations cause HHT is haploinsufficiency.

30 In primary CLL cells, HHT induced significant apoptosis independent of the pro

31 ts were 18 to 70 years old and had confirmed HHT, severe liver involvement, and a high cardiac index

32 Eighty consecutive HHT patients were randomized and treated in the phase 2

33 utation predisposes an individual to develop HHT-associated vascular lesions.

34 linical criteria for HHT and had experienced HHT-related epistaxis with an Epistaxis Severity Score o

35 t patients who met the clinical criteria for HHT and had experienced HHT-related epistaxis with an Ep

36 re undiscovered mutations in other genes for HHT and possibly vascular disorders with overlapping phe

37 ng pathways, the pathogenetic mechanisms for HHT remain elusive.

38 iber, and support a novel two-step model for HHT-associated AVM development in which pathological art

39 Optimal treatment for HHT-related epistaxis is uncertain.

40 al with national recruitment from the French HHT Network.

41 We found that human liver microsomes have HHT/MDA synthase activity that is concentration-dependen

42 ted individuals clinically suspected to have HHT was investigated with the use of exome and Sanger se

43 Homoharringtonine (HHT) is a novel plant alkaloid that produced a complete

44 Homoharringtonine (HHT) is a plant alkaloid that inhibits the elongation ph

45 a combination regimen of homoharringtonine (HHT) and low-dose cytarabine (ara-C) in patients with Ph

46 hibitor of transcription, homoharringtonine (HHT), a protein synthesis inhibitor, and imatinib were u

47 we studied the 12-hydroxyheptadecatrienoate (HHT)/malondialdehyde (MDA) synthase activity of human li

48 ng BMP9-unresponsive ALK1 HHT mutants and in HHT patient blood outgrowth ECs.

49 The North American Study of Epistaxis in HHT was a double-blind, placebo-controlled randomized cl

50 leeding rate at presentation was observed in HHT (p = 0.069) and an increased rate of giant venous po

51 nvironmental factors that cause pathology in HHT type 2 patients.

52 osing has therefore therapeutic potential in HHT.

53 histological similarities to lesions seen in HHT patients.

54 spleen and brain), similar to those seen in HHT patients.

55 ls similar to vascular malformations seen in HHT patients.

56 in the cytoplasmic connecting link to IVS5 (HHT-5371) and in IVS5 transmembrane segment (HHT-5411) w

57 For mutants HHT-5411 and HHT-5371, the residual current appeared ass

58 stigating the simultaneous administration of HHT and IFN-alpha, as well as that of HHT and low-dose c

59 In this study, six courses of HHT were administered to 90 patients with early chronic

60 vide a rationale for clinical development of HHT in CLL as single agent or in combinations.

61 s aged 18 years or older with a diagnosis of HHT were recruited from 5 French centers from April 2014

62 CVRL1 (4.3 +/- 6%) leading to a diagnosis of HHT, and 14 in RASA1 (60.9 +/- 14.4%) leading to a diagn

63 s the precipitating event in the etiology of HHT.

64 15% of individuals with clinical features of HHT do not have mutations in these genes, suggesting tha

65 mations mimicking all pathologic features of HHT.

66 k to chromosome 12q13 or in which linkage of HHT to chromosome 9q33 had been excluded.

67 insights into the pathogenetic mechanisms of HHT and potential therapeutic approaches.

68 nodepleted postnatal retina-a mouse model of HHT vascular pathology-tacrolimus activated endothelial

69 , noninvasive, reliable, and robust model of HHT vascular pathology.

70 1 signaling pertinent to the pathogenesis of HHT and suggest that HHT might not be a TGF-beta subfami

71 e 2 receptors play a role in pathogenesis of HHT is unknown.

72 that may be relevant to the pathogenesis of HHT vascular lesions.

73 mportant questions about the pathogenesis of HHT.

74 understanding of the molecular pathology of HHT [corrected] in particular and to angiogenesis in gen

75 ata demonstrate that accelerated recovery of HHT-5371 and HHT-5411 decreased accumulation of these ch

76 The combination regimen of HHT and ara-C is effective and safe in patients with CML

77 TGFBM2 locus influence clinical severity of HHT, [corrected] as assessed by development of pulmonary

78 ion of HHT and IFN-alpha, as well as that of HHT and low-dose cytosine arabinoside in patients failin

79 we hypothesize that phenotypic variation of HHT is not related to a particular ENG mutation.

80 Contrary to the current view of HHT as venous disease, our findings suggest that the art

81 VFs, and show the predominance of RASA1 over HHT mutations.

82 Patients received HHT 2.5 mg/m(2) by continuous infusion daily for 5 days

83 ere seen in 66% of the patients who received HHT and IFN-alpha compared with 61% of the historical co

84 After receiving HHT, patients required lower doses of IFN-alpha to maint

85 In addition, we find that 12-S-HHT, but not 16:4(n-3), functions via leukotriene B4 rec

86 or chemical inhibition of BLT2 prevents 12-S-HHT-mediated resistance.

87 HHT-5371) and in IVS5 transmembrane segment (HHT-5411) with both diltiazem and verapamil.

88 Hereditary haemorrhagic telangiectasia (HHT) [corrected] is a vascular dysplasia syndrome caused

89 d in hereditary haemorrhagic telangiectasia (HHT), atherosclerosis, tumorigenesis and immunomodulatio

90 sia, hereditary haemorrhagic telangiectasia (HHT), wherein arterial and venous beds fail to remain di

91 with hereditary hemorrhagic telangiectasia (HHT) and cardiac failure is liver transplant.

92 ling: hereditary hemorrhagic telangiectasia (HHT) and cerebral cavernous malformation (CCM).

93 ed in hereditary hemorrhagic telangiectasia (HHT) and vascular remodeling, acting via the HHT target

94 Hereditary hemorrhagic telangiectasia (HHT) is a highly debilitating and life-threatening genet

95 Hereditary hemorrhagic telangiectasia (HHT) is a potentially life-threatening genetic vascular

96 Hereditary hemorrhagic telangiectasia (HHT) is a vascular dysplasia characterized by the inappr

97 Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characte

98 Hereditary hemorrhagic telangiectasia (HHT) is an autosomal-dominant disorder characterized by

99 Hereditary hemorrhagic telangiectasia (HHT) is an inherited autosomal dominant vascular dysplas

100 ic of hereditary hemorrhagic telangiectasia (HHT), a disease caused by mutations in activin-like kina

101 cause hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disorder of localized angiod

102 uding hereditary hemorrhagic telangiectasia (HHT), cancer, atherosclerosis and immunomodulation.

103 Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is an autosomal domi

104 In hereditary hemorrhagic telangiectasia (HHT), the hallmark vascular lesion is termed an arteriov

105 Hereditary hemorrhagic telangiectasia (HHT), the most common inherited vascular disorder, is ca

106 gated hereditary hemorrhagic telangiectasia (HHT), which is caused by mutations in TGFbeta/bone morph

107 ermed hereditary hemorrhagic telangiectasia (HHT), which is characterized by recurrent nosebleeds, mu

108 sease hereditary hemorrhagic telangiectasia (HHT).

109 auses hereditary hemorrhagic telangiectasia (HHT).

110 on of hereditary hemorrhagic telangiectasia (HHT).

111 with hereditary hemorrhagic telangiectasia (HHT).

112 cause hereditary hemorrhagic telangiectasia (HHT, or Rendu-Osler-Weber syndrome), clinical evaluation

113 with hereditary hemorrhagic telangiectasia (HHT; also known as Osler-Weber-Rendu disease).

114 Genetic studies have demonstrated that HHT can be caused by loss-of-function mutations in the g

115 to the pathogenesis of HHT and suggest that HHT might not be a TGF-beta subfamily disease.

116 These data suggest that HHT pathogenesis involves disruption of a complex networ

117 MP9-regulated proteins that could affect the HHT phenotype.

118 HHT) and vascular remodeling, acting via the HHT target genes, endoglin and ALK1.

119 exposed to ara-C and 11% had been exposed to HHT.

120 tes with BMP9/10 blocking antibodies lead to HHT-like vascular defects in the postnatal retinal angio

121 Two genes are linked to HHT: endoglin (ENG) in HHT1 and activin receptor-like ki

122 ed as the most likely ALK1 ligand related to HHT, yet the identity of the physiologic ALK1 ligand rem

123 lvement, and a high cardiac index related to HHT.

124 Similar to HHT patients, the mice also exhibited gastrointestinal b

125 adjusted from 6.6 to 7.5, high heat treated (HHT, 95 degrees Cx2min) or held unheated for 1h, re-adju

126 es were identical with HHT plus ara-C versus HHT alone, but the survival was significantly longer wit

127 Greater synergy was observed when HHT and imatinib were given sequentially compared with s

128 ty-two percent of patients achieved CHR with HHT; CG responses were observed in 60% and were major in

129 tions were found in six of six families with HHT either shown to link to chromosome 12q13 or in which

130 Response rates were identical with HHT plus ara-C versus HHT alone, but the survival was si

131 ly syndrome that has phenotypic overlap with HHT.

132 In this preliminary study of patients with HHT associated with severe hepatic vascular malformation

133 In patients with HHT, a bevacizumab nasal spray treatment of 3 administra

134 Among patients with HHT, there were no significant between-group differences

135 ious study group of 73 patients treated with HHT alone.