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 tact activation) and regulated by the serpin C1 inhibitor.

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

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

8 ear to involve genes for proteins other than C1-inhibitor.

9 ing pathway-inhibitor-3, and Plasma-protease-C1-inhibitor.

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

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

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

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

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

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

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

17 most HAE cases are caused by reduced plasma C1-inhibitor activity, HAE has been linked to lysine/arg

18 Most patients with HAE have low plasma C1-inhibitor activity, leading to increased generation o

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

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

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

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

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

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

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

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

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

28 entifies A1 as a new class of small-molecule C1s inhibitor and lays the foundation for development of

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

30 a, FXIa, and kallikrein with antithrombin or C1 inhibitor, and prevented cleavage and consumption of

31 ransferrin, recombinant erythropoietin-beta, C1-inhibitor, and glutathione during the anhepatic and r

32 way proteases nor any protease controlled by C1-inhibitor are required for MASP-3 activation.

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

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

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

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

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

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

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

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

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

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

43 outcomes after transplantation is the use of C1 inhibitor (C1-INH), which blocks the first step in bo

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

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

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

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

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

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

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

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

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

53 b to twice-weekly subcutaneous injections of C1-inhibitor (C1-INH), she had no attacks requiring addi

54 ks, most frequently due to absent or reduced C1 inhibitor (C1INH) activity.

55 is regulated by the ambient concentration of C1 inhibitor (C1INH) and prolylcarboxypeptidase (PRCP).

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

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

58 C1 inhibitor (C1INH) is a multifunctional serine proteas

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

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

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

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

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

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

65 mutations in the SERPING1 gene encoding the C1 inhibitor (C1INH) that leads to plasma deficiency, re

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

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

68 ations in the SERPING1 gene that encodes the C1 inhibitor (C1INH), a serine protease inhibitor (serpi

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

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

71 to plasma deficiency of the encoded protein C1 inhibitor (C1INH).

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

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

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

75 vels for regulators of the kallikrein-kinin (C1-inhibitor), coagulation (thrombomodulin, endothelial

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

91 C1 inhibitor concentrates and fresh frozen plasma are av

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

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

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

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

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

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

98 ients aged 12 years or older with HAE due to C1 inhibitor deficiency and at least 2 investigator-conf

99 C1 inhibitor deficiency and patients with bowel edema on

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

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

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

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

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

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

106 io, patients with hereditary angioedema with C1 inhibitor deficiency to receive four subcutaneous dos

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

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

109 nity including autoinflammatory diseases and C1 inhibitor deficiency, and 33 with symptomatic SID.

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

111 ctic treatment of hereditary angioedema with C1 inhibitor deficiency.

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

113 Angioedema due to acquired C1-inhibitor deficiency (AAE-C1INH) is a rare disorder c

114 reditary angioedema (HAE) comprises HAE with C1-inhibitor deficiency (C1-INH-HAE) and HAE with normal

115 Angioedema due to acquired C1-inhibitor deficiency is a very rare but serious disea

116 Hereditary angioedema (HAE) due to C1-inhibitor deficiency or dysfunction is a rare genetic

117 d prevent angioedema attacks due to acquired C1-inhibitor deficiency.

118 tic treatment for angioedema due to acquired C1-inhibitor deficiency.

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

120 lly caused by deficiency or mutations in the C1-inhibitor gene, SERPING1, a member of the Serpin fami

121 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

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

123 to highlight the burden of disease caused by C1-inhibitor HAE across the Asia-Pacific region, conside

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

125 Hereditary angioedema (HAE) with normal C1 inhibitor (HAE-nC1INH), including HAE arising from F1

126 cy of functional C1 inhibitor or with normal C1 inhibitor (HAEnCI).

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

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

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

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

131 Similarly, well-conducted studies with C1 inhibitors in sensitized recipients yielded disappoin

132 (sutimlimab, previously TNT009), a targeted C1s inhibitor, in 10 subjects with active or past bullou

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

134 C1 inhibitor is a multifunctional protein produced in th

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

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

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

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

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

140 overwhelm the normal inhibitory function of C1 inhibitor, leading to increased HK cleavage.

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

142 transplantation led to normalization of the C1 inhibitor level and function.

143 ally confirmed hereditary angioedema and low C1 inhibitor levels (but without previous episodes of an

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

145 dolescents and patients with HAE with normal C1 inhibitor levels; and tolerability has been demonstra

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

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

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

149 cold agglutinin disease, primarily with the C1s inhibitor of the classical complement pathway sutiml

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

151 ated with a genetic deficiency of functional C1 inhibitor or with normal C1 inhibitor (HAEnCI).

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

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

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

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

156 bitor (icatibant acetate) and plasma-derived C1 inhibitor (pdC1INH) led to an improvement in lung com

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

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

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

160 Hereditary angioedema (HAE), caused by C1 inhibitor protein deficiency, was recently shown to b

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

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

163 tration approved, identifying small-molecule C1s inhibitors remains a priority.

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

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

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

167 are caused by mutations in the gene encoding C1 inhibitor, SERPING1.

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

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

170 The complement C1s inhibitor sutimlimab is an emerging option in the se

171 nd represent a distinct mechanistic class of C1 inhibitors that protect the spirochete from antibody-

172 t common cause is reduced plasma activity of C1 inhibitor, the main regulator of the proteases plasma

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

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

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

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

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

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