ライフサイエンスコーパス: C1-inhibitor

1 plement complex), and regulators (total CFH, C1 inhibitor).

2 function of plasma trough concentrations of C1 inhibitor.

3 disorder caused by deficiency of functional C1 inhibitor.

4 This was inhibited by the C1 inhibitor.

5 ma is caused by a heterozygous deficiency of C1 inhibitor.

6 a useful technique to facilitate studies on C1-inhibitor.

7 sed by partial absence of the plasma protein C1-inhibitor.

8 ls in which prophylactic infusions of either C1 inhibitor (25 plasma units per kilogram of body weigh

9 C1 inhibitor, a protein known to inhibit activated C1s,

10 edema is an autosomal-dominant deficiency of C1 inhibitor--a serpin inhibitor of kallikrein, C1r, C1s

11 hat peptides from platelet basic protein and C1 inhibitor achieved both 100% sensitivity and 100% spe

12 injection, resulted in functional levels of C1 inhibitor activity that would be expected to provide

13 l elastase release, possibly by accelerating C1 inhibitor activity.

14 Point mutations of antithrombin, C1 inhibitor, alpha(1)-antichymotrypsin, and heparin cof

15 ly history and the measurement of functional C1 inhibitor and C4 levels.

16 lted in close to normal functional levels of C1 inhibitor and C4.

17 alphaFXIIa-polyP70 complex was modulated by C1 inhibitor and histidine-rich glycoprotein, but not pl

18 s from a congenital deficiency of functional C1 inhibitor and is characterized by episodic bouts of e

19 significantly increased concentrations of C1-C1 inhibitor and kallikrein-C1 inhibitor complexes.

20 e presence of "complete" serum that contains C1-inhibitor and alpha(2)-macroglobulin.

21 hereditary angioedema (HAE) are deficient in C1-inhibitor and consequently exhibit excessive bradykin

22 onization of bacteria with a mixture of pure C1-inhibitor and/or alpha(2)-macroglobulin added togethe

23 proteinase inhibitor (alpha(1)-antitrypsin), C1 inhibitor, and most efficiently by antithrombin-hepar

24 gulated in healthy individuals by the serpin C1-inhibitor, but individuals with hereditary angioedema

25 A dysfunctional C1 inhibitor (C1 INH) from a family in whom the proposit

26 C1 inhibitor (C1 INH) is the major inhibitor of the prot

27 ion of C1s with its natural pseudosubstrate, C1 inhibitor (C1 inh), and promotion of proteolytic acti

28 We report six patients with acquired C1 inhibitor (C1-inh) deficiency associated with serum C

29 C1 inhibitor (C1-INH) is known to form complexes with th

30 reaction is stoichiometric and inhibited by C1 inhibitor (C1-INH) or corn trypsin inhibitor.

31 C1 inhibitor (C1-INH) regulates several pathways which c

32 II are caused by a functional deficiency of C1 inhibitor (C1-INH), leading to overproduction of brad

33 ts of a selective PK inhibitor, ASP-440, and C1 inhibitor (C1-INH), the primary physiological inhibit

34 C1 inhibitor (C1-INH, Berinert) inhibits the classical a

35 reditary angioedema due to the deficiency of C1-inhibitor (C1-INH) (HAE-C1-INH).

36 arin on inhibition of FXIa by the inhibitors C1-inhibitor (C1-INH) and antithrombin III (ATIII).

37 We studied the kinetics of C1-inhibitor (C1-INH) and other complement parameters in

38 (fXIa) by the serpins antithrombin (AT) and C1-inhibitor (C1-INH) by more than 2 orders of magnitude

39 Hereditary angioedema (HAE) caused by C1-inhibitor (C1-INH) deficiency (HAE-C1-INH) is a poten

40 attacks of hereditary angioedema (HAE) with C1-inhibitor (C1-INH) deficiency.

41 ry angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), leads to unpredictable edema of s

42 rate S2366, and to undergo inhibition by the C1-inhibitor (C1-INH), protein Z dependent protease inhi

43 Hereditary angioedema (HAE) with normal C1 inhibitor (C1Inh) associated with the c.983C>A and c.

44 Heterozygosity for C1 inhibitor (C1INH) deficiency results in hereditary an

45 Plasma C1 inhibitor (C1INH) is a natural inhibitor of complemen

46 C1 inhibitor (C1INH) is beneficial in animal models of e

47 C1 inhibitor (C1INH) prevents endotoxin shock in mice vi

48 The C1 inhibitor (C1INH) promoter is unusual in two respects

49 C1 inhibitor (C1INH) protects mice from lethal Gram-nega

50 Controlling prekallikrein activation by C1 inhibitor (C1Inh) represents the most essential mecha

51 C1 inhibitor (C1INH), a member of the serine proteinase

52 The C1 inhibitor (C1INH), a plasma complement regulatory pro

53 Dysfunctional C1 inhibitor (C1INH)-Ta is a naturally occurring mutant

54 leads to enhanced synthesis and secretion of C1 inhibitor (C1inh).

55 a (HAE) caused by a deficiency of functional C1-inhibitor (C1INH) becomes clinically manifest as atta

56 Human C1-inhibitor (C1INH) is a multifunctional protease inhib

57 Angioedema due to hereditary deficiency of C1 inhibitor causes temporarily disability.

58 vation and increased formation of kallikrein-C1 inhibitor complexes, without Factor XIa activation an

59 entrations of C1-C1 inhibitor and kallikrein-C1 inhibitor complexes.

60 ctic twice-weekly injections of nanofiltered C1 inhibitor concentrate (1000 units) with placebo durin

61 Plasma-derived human C1 inhibitor concentrate (pdhC1INH) is preferred for acu

62 ebo (P<0.001); the subjects who received the C1 inhibitor concentrate also had significant reductions

63 Infusions of a vapor-heated C1 inhibitor concentrate are a safe and effective means

64 ack was 2 hours in the subjects treated with C1 inhibitor concentrate but longer than 4 hours in thos

65 of attacks per 12-week period was 6.26 with C1 inhibitor concentrate given as prophylaxis, as compar

66 o randomized trials to evaluate nanofiltered C1 inhibitor concentrate in the management of hereditary

67 We evaluated the effectiveness of a C1 inhibitor concentrate in the prevention and treatment

68 When used for prophylaxis, nanofiltered C1 inhibitor concentrate reduced the frequency of acute

69 The infusions of C1 inhibitor concentrate resulted in close to normal fun

70 cts with hereditary angioedema, nanofiltered C1 inhibitor concentrate shortened the duration of acute

71 The first study compared nanofiltered C1 inhibitor concentrate with placebo for treatment of a

72 medications including icatibant (N = 3) and C1-inhibitor concentrate (N = 6) were used in 13 cases.

73 C1 inhibitor concentrates and fresh frozen plasma are av

74 ttenuated androgens, anti-fibrinolytics, and C1 inhibitor concentrates are used for long-term and pre

75 ients aged 13 years or older with functional C1-inhibitor concentrations of less than 50% of normal a

76 lished to date on hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) have focused on adu

77 Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare inherited

78 Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by

79 atients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH).

80 C1 inhibitor deficiency and patients with bowel edema on

81 radykinin is a major mediator of swelling in C1 inhibitor deficiency as well as the angioedema seen w

82 Hereditary angioedema due to C1 inhibitor deficiency is characterized by recurrent ac

83 Hereditary angioedema with C1 inhibitor deficiency is characterized by recurrent, u

84 Hereditary angioedema (HAE) due to C1 inhibitor deficiency manifests as recurrent swelling

85 to patients with hereditary angioedema with C1 inhibitor deficiency reduced cleavage of high-molecul

86 rom patients with hereditary angioedema with C1 inhibitor deficiency to levels approaching that from

87 Patients with hereditary angioedema with C1 inhibitor deficiency were randomly assigned in a 2:1

88 roundtable discussion took place at the 6th C1 Inhibitor Deficiency Workshop (May 2009, Budapest, Hu

89 panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.

90 eter update: Hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzy

91 with HAE-N that is not seen in patients with C1 inhibitor deficiency.

92 ctic treatment of hereditary angioedema with C1 inhibitor deficiency.

93 Perfusion of C1-inhibitor-depleted plasma over glomerular endothelial

94 roup (36 min, CI: 26-46) (P<0.01) and in the C1 inhibitor group (23 min, CI: 21-25) (P<0.05), and pul

95 A total of 68 subjects (35 in the C1 inhibitor group and 33 in the placebo group) were giv

96 Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disablin

97 ptor deficient mice, and Crry-Ig and soluble C1 inhibitor have both been shown to have therapeutic ef

98 gory) and could be corrected by supplemental C1 inhibitor in 4 of them.

99 In this study, we analyzed the expression of C1 inhibitor in fibroblasts obtained from a skin biopsy

100 betic individuals, and a subset of proteins, C1 inhibitor in particular, were exceptionally good disc

101 improvement in symptoms was shorter for the C1 inhibitor infusions than the placebo infusions (55 vs

102 Hereditary angio-oedema (HAE) with normal C1 inhibitor is associated with heterozygous mutations i

103 C1-inhibitor is a proteinase inhibitor in the serpin fam

104 C1-inhibitor is the main inhibitor of the kinin system.

105 produce monomer, dimer, and polymer forms of C1-inhibitor is useful for studies investigating the con

106 New agents such as recombinant C1 inhibitor, kallikrein inhibitors, and bradykinin inhi

107 Quantitative or qualitative deficiency of C1 inhibitor leads to the generation of vasoactive media

108 Hereditary angioedema with normal C1 inhibitor levels (HAE-N) is associated with a Factor

109 e I hereditary angioedema, reduced levels of C1 inhibitor may be due in part to increased turnover an

110 a patient was recently described in whom the C1 inhibitor mutation consisted of a 20-bp duplication o

111 an blood unmodified (n=7) or pretreated with C1 inhibitor (n=5) or soluble complement receptor type 1

112 ic disease usually caused by mutation in the C1 inhibitor or the coagulation Factor XII gene.

113 ffect significantly decreased by addition of C1-inhibitor or B1 receptor-antagonist.

114 of TPA activity, TPA antigen, TPA/PAI-1, TPA/C1 inhibitor, PAI-1 activity, and PAI-1 antigen over a 4

115 Drug treatment comprised plasma-derived C1 inhibitor (pdC1-INH) for acute swelling attacks and p

116 kly intravenous injections of plasma-derived C1-inhibitor (pdC1-INH) has been established as an effec

117 after infusions of either 25 plasma units of C1 inhibitor per kilogram (55 infusions in 11 patients)

118 e 2 trial, the use of CSL830, a nanofiltered C1 inhibitor preparation that is suitable for subcutaneo

119 lation and this led to excess consumption of C1 inhibitor protein (C1 INH), a major regulator of both

120 d turnover and decreased synthesis of normal C1 inhibitor protein.

121 ncy (type I) or dysfunction (type II) of the C1 inhibitor protein.

122 ibitor, our results suggest that efficacy of C1-inhibitor replacement therapy might not be a direct f

123 ared with placebo, prophylactic infusions of C1 inhibitor resulted in significantly lower daily sympt

124 Recombinant human C1 inhibitor (rhC1INH) for on-demand treatment of heredi

125 and the reduction of these microvesicles by C1-inhibitor should be explored as a potential treatment

126 eased extracellular production of the normal C1 inhibitor, suggesting either decreased secretion or i

127 ligand until it was treated with EDTA or the C1 inhibitor to remove the C1r2C1s2 complex from C1, lea

128 dema, the prophylactic use of a subcutaneous C1 inhibitor twice weekly significantly reduced the freq

129 superfamily, protein nexin 1, alpha1-PI, and C1-inhibitor, was unaffected indicating that the RRHR mo

130 SERPING1 encodes the C1 inhibitor, which has a crucial role in inhibition of

131 king PN1 a far better inhibitor of FXIa than C1 inhibitor, which is the only other SERPIN known to si

132 rticularly hereditary angioedema with normal C1 inhibitor with a factor XII mutation.