ライフサイエンスコーパス: factor H

1 ils compared with the major allele (Val62 in factor H).

2 Lchain restriction during reactivity against factor H.

3 ent cascade by interacting with C1q and with Factor H.

4 d to more potent inhibition of complement by factor H.

5 nt receptor 1 but did not perturb binding to factor H.

6 ment cascade C1q, C6, and C8; and complement factor H.

7 s, such as C-reactive protein and complement factor H.

8 own to bind the complement regulator protein factor H.

9 inding of inhibitors C4b-binding protein and factor H.

10 llular matrix, recruited functionally active factor H.

11 t of C3b or iC3b to attract large amounts of factor H.

12 , but it also binds the complement inhibitor factor H.

13 degradation in the presence of the cofactor Factor H.

14 Hic differs from binding to vitronectin and factor H.

15 in on PGA formation are tightly regulated by Factor H.

16 ivation in individuals with fully functional factor H.

17 n tissues suppresses regulation of the AP by factor H.

18 we consider moss-produced recombinant human factor H a promising pharmaceutical product for therapeu

19 eceptor 2 linked to the inhibitory region of factor H, a molecule directly targeting complement-activ

20 Endogenous factor H, a potent inhibitor of the alternative pathway,

21 Unlike factor H, a potent negative regulator of complement C3 a

22 Factor H acts as a cofactor for factor I-mediated cleava

23 Ten loci in 7 AMD genes [complement factor H, age-related maculopathy susceptibility 2/high-

24 In contrast to C3b or Factor H alone, the solubility of the central C3b-Factor

25 We now document that factor H also binds to tubular HS, although to a differe

26 omplement inhibitors C4b-binding protein and factor H also interact with dying cells, most likely to

27 tein (C4BP; classical pathway inhibitor) and factor H (alternative pathway [AP] inhibitor).

28 osporine-induced microparticles did not bind factor H, an alternative pathway regulatory protein pres

29 rmore, IAPP also bound complement inhibitors factor H and C4b-binding protein (C4BP).

30 rtain mutations and autoantibodies affecting factor H and CFHR1, which could result in an enhanced lo

31 ctions of the negative complement regulators Factor H and complement receptor 1 with C3b.

32 t regulatory factors C4b-binding protein and factor H and confirmed that the ability to bind at least

33 in A (sIgA) but not the complement regulator factor H and did not decrease C3b deposition on the pneu

34 y activity similar to that of plasma-derived factor H and efficiently blocked LPS-induced alternative

35 product that lacks the recognition domain of factor H and exhibits impaired cell surface complement r

36 ans uses human complement regulators such as factor H and factor H-like protein 1 (FHL-1) for immune

37 n 1 (CFHR1) bind to PTX3 and that PTX3-bound factor H and factor H-like protein 1 maintain their comp

38 Factor H and FHL-1 displayed complement-regulatory activ

39 annexin A2 impaired complement regulation by factor H and increased complement activation on renal ce

40 In conclusion, factor H and properdin interact with different HS epitop

41 ent nonoverlapping epitopes on HS/heparin by factor H and properdin.

42 S patients inhibited the interaction between factor H and PTX3, and five autoantibodies also inhibite

43 tion of C3b only occurred with the cofactors factor H and soluble CR1 but not with CD46.

44 context, the balance between the actions of factor H and the FHR proteins determines the degree of c

45 In mice deficient in complement factor H and transgenic for human CR1, soluble CR1 thera

46 gous to the complement inhibitory domains of factor H and, accordingly, has no significant complement

47 y in aged (12 months) mice deficient in both factors H and C3 (CFH(-/-).C3(-/-)), CFH alone (CFH(-/-)

48 n the presence of the complement regulators (factors H and I), and this degradation results in lower

49 tio; the C3b portion was rapidly degraded by factors H and I.

50 ies on the interaction of C3b with Factor B, Factor H, and complement receptor 1.

51 the alternative pathway negative regulator, Factor H, and how aiming to understand these interaction

52 Patients with MPGN had normal levels of factor H, and structural analysis of the mutant revealed

53 or simultaneous interaction with both Vn and factor H, and that it recognized the C-terminal part of

54 tic complement abnormalities/anti-complement factor H antibodies, which paved the way to treatment wi

55 Mutations and genetic variations of factor H are associated with several AP-mediated disease

56 les of the factor H-related proteins, unlike factor H, are incompletely understood.

57 cay induced by decay-accelerating factor and factor H, as assayed by surface plasmon resonance.

58 Similarly, seven of nine analyzed anti-factor H autoantibodies isolated from aHUS patients inhi

59 l surface protein, possesses vitronectin and factor H binding activity.

60 thin outer membrane P5 affected the level of factor H binding between individual strains.

61 A novel mechanism of factor H binding is presented in which YadA exploits rec

62 spectrometry, we identified annexin A2 as a factor H binding partner.

63 st sheaths presenting Neisseria meningitidis factor H binding protein (fHbp) antigen were functional

64 is not known to bind human FH, and a mutant factor H binding protein (FHbp) antigen with a >50-fold

65 Meningococcal vaccines containing factor H binding protein (fHbp) are in clinical developm

66 isolates, we identified four (16%) with high factor H binding protein (FHbp) expression that were res

67 Meningococcal factor H binding protein (fHbp) is a human species-speci

68 Factor H binding protein (fHbp) is a lipoprotein of Neis

69 Factor H binding protein (FHbp) is part of two vaccines

70 iques, we generated a series of mAbs against factor H binding protein (fHbp), a key virulence factor

71 Factor H binding protein (fHbp), a virulence factor whic

72 ride, lipooligosaccharide (LOS) sialic acid, factor H binding protein (fHbp), and neisserial surface

73 icensed; both vaccines contain meningococcal factor H binding protein (fHbp).

74 test strains expressing vaccine-heterologous factor H binding protein variants: PMB80 (A22), PMB2001

75 MenB-FHbp (factor H binding protein), a serogroup B meningococcal (

76 for capsule biosynthesis, the expression of factor H binding protein, and sialylation of lipopolysac

77 Bivalent rLP2086 is a recombinant factor H binding protein-based vaccine approved in the U

78 inding to tubular HS, with a minor effect on factor H binding to tubular HS.

79 examined both proteins in animal infection, factor H binding, and serum sensitivity assays.

80 d complement resistance through varied human factor H binding, that may affect invasiveness in childr

81 cocci to express similar amounts of the same factor H-binding protein (fHbp; a key component of group

82 ere closely related to the vaccine antigens (factor H-binding protein [fHbp] and neisserial heparin-b

83 ty of the nitrite reductase gene (aniA), the factor H-binding protein gene (fHbp), and the capsule bi

84 a licensed meningococcal B vaccine targeting factor H-binding protein.

85 strains that expressed vaccine-heterologous factor H-binding proteins representative of meningococca

86 Factor H binds to Lig proteins via short consensus repea

87 We conclude that factor H binds to VWF and may modulate cleavage of VWF b

88 We found that factor H binds to VWF in plasma, to plasma-purified VWF,

89 d analysis showed that FHR3, but not FHR1 or factor H, blocked B cell activation by the BCR corecepto

90 blocks all pathways at C3 activation) or CR2-factor H (blocks alternative pathway) was highly protect

91 PP fibrils and C1q, C3d, as well as C4BP and factor H but not membrane attack complex.

92 solate, and reported that Hif interacts with factor H by expressing protein H (PH).

93 n the presence of several cofactors, such as factor H, C4b-binding protein, complement receptor 1, an

94 enetic risk was based on Y402H in complement factor H (CFH) and A69S in age-related maculopathy susce

95 enotyped for polymorphisms in the complement factor H (CFH) and age-related maculopathy susceptibilit

96 r at chromosome 1q32 contains the complement factor H (CFH) and five complement factor H-related (CFH

97 nd genetic variants in inhibitory complement factor H (CFH) are also features of both ARMD and TMA, w

98 Genetic variations in complement factor H (CFH) confer greater risk for age-related macul

99 Rare variants in the complement factor H (CFH) gene and their association with age-relat

100 le why non-coding variants in the complement factor H (CFH) gene are more strongly associated with ag

101 ocus on genetic variations in the complement Factor H (CFH) gene cluster and CFH autoantibodies in si

102 of the Y402H risk variant in the complement factor H (CFH) gene developed neovascular AMD 2.8 (95% C

103 t amino acid position 402) in the complement factor H (CFH) gene have a pharmacogenetics effect on th

104 chromosome 1q31.1 containing the complement factor H (CFH) gene was strongly associated with serum M

105 athy susceptibility 2 (ARMS2) and complement factor H (CFH) genotypes, and other factors, mean IMT wa

106 Complement factor H (CFH) is a central regulator of the complement

107 Complement factor H (CFH) is a major susceptibility gene for age-re

108 Complement factor H (CFH) is a negative regulator of the alternativ

109 Complement factor H (CFH) is an important regulatory protein in the

110 62V substitutions in the gene for complement factor H (CFH) is strongly associated with risk of AMD.

111 loci and 6 additional SNPs at the complement factor H (CFH) locus.

112 tion is efficiently suppressed by complement factor H (CFH) on self-surfaces but not on foreign surfa

113 d presence of risk alleles of the complement factor H (CFH) or age-related maculopathy susceptibility

114 Complement factor H (CFH) regulates complement activation in host t

115 .1 years, individuals with 1 or 2 complement factor H (CFH) risk alleles derived maximum benefit from

116 and AMD-susceptibility genotypes Complement Factor H (CFH) RS1061170 and Age Related Maculopathy Sus

117 Two loci in complement factor H (CFH) were included in a risk score to determin

118 us (CMV) immunoglobulin (Ig)G and complement factor H (CFH) Y402H genotype.

119 These include complement factor H (CFH), a negative regulator of C3 activation.

120 Ten genetic loci in 7 genes [complement factor H (CFH), age-related maculopathy susceptibility 2

121 Genetic variants of complement factor H (CFH), C3, C2, and FB associated with increased

122 filtered for rare variants in the complement factor H (CFH), complement factor I (CFI), complement C9

123 ysiological complement inhibitor, complement factor H (CFH), for ligand binding.

124 ariants of the CFH gene, encoding complement factor H (CFH), show strong association with age-related

125 the negative complement regulator complement factor H (CFH), thereby inhibiting the alternative pathw

126 ijacking a host-immune regulator, complement factor H (CFH), to the bacterial surface.

127 comparison of hemopexin (HPX) and complement factor H (CFH), two liver-secreted glycoproteins, in hea

128 , smoking status, presence of the complement factor H (CFH)-rs1061170 and age-related maculopathy sus

129 roteins or autoantibodies against complement factor H (CFH).

130 athy susceptibility 2 (ARMS2) and complement factor H (CFH).

131 four retinal genes interact with Complement Factor H (CFH).

132 and rare, in the coding region of complement factor H (CFH).

133 hat, in contrast to the complement inhibitor factor H, CFHR4 acts as an enhancer of opsonization by p

134 ecific for the central complement regulator, factor H, combined with a homozygous deficiency, mostly

135 The removal of the C3b-Factor H complex by zinc explains the reduced C3u/C3b in

136 u(1032) salt bridge is essential for the C3b-Factor H complex during the regulatory control of C3b, t

137 r H alone, the solubility of the central C3b-Factor H complex was much reduced at 60 muM zinc and eve

138 Factor H contacts Lpd via short consensus repeats 7 and

139 Substitution with biologically active factor H could potentially treat a variety of diseases t

140 , C. albicans cell extract was absorbed to a factor H-coupled matrix.

141 or showed a nonsense CFH mutation leading to factor H deficiency.

142 y decreased in the absence of either OlpA or factor H, demonstrating that this inhibition of the alte

143 nd there was a differential binding of human factor H, depending on invasiveness.

144 f an improved moss-derived recombinant human factor H devoid of potentially immunogenic plant-specifi

145 Coincubation of properdin and factor H did not hamper HS/heparin binding of one anothe

146 to bind human complement regulatory protein factor H directly, thereby, preventing complement factor

147 Thus, although endogenous factor H does play a role in limiting the development of

148 reely into solution when the five complement factor H domains are bound within C3b.

149 Factor H enhanced ADAMTS-13-mediated cleavage of recombi

150 In this study, we show that native factor H, factor H-like protein 1, and factor H-related

151 DAF) expression on endothelial cells, giving Factor H (FH) a major role in complement regulation.

152 Factor H (FH) and C4-binding protein (C4BP), which conco

153 Factor H (fH) and properdin both modulate complement; ho

154 nomic disorders affecting the genes encoding factor H (fH) and the 5 factor H related proteins have b

155 mic aberrations affecting the genes encoding factor H (FH) and the five FH-related proteins (FHRs) ha

156 nition domains 19-20 of complement regulator factor H (FH) are strongly associated with aHUS, but the

157 s to be efficiently down-regulated by plasma factor H (FH) as exemplified by various diseases caused

158 Factor H (fH) binding protein (fHbp) is part of vaccines

159 roups), Neisserial surface protein A (NspA), factor H (fH) binding protein (fHbp), and lipooligosacch

160 Further, we show that complement factor H (FH) binds mCRP to dampen its proinflammatory a

161 Complement regulatory protein factor H (fH) binds modified host proteins and lipids to

162 the complement alternative pathway regulator factor H (FH) competes with factor B for C3b binding; ho

163 binding of the alternative pathway inhibitor factor H (FH) could be one mechanism causing variations

164 nt inhibitors C4b-binding protein (C4BP) and factor H (FH) exert a tight regulation of the classical/

165 controlled by regulatory proteins, including factor H (FH) in plasma and membrane cofactor protein (M

166 3dg and enhanced competition with complement factor H (FH) in surface plasmon resonance (SPR) studies

167 Complement factor H (FH) inhibits complement activation and interac

168 Factor H (FH) is a key alternative pathway regulator tha

169 Complement factor H (fH) is a plasma protein that regulates activat

170 Factor H (fH) is an endogenous negative regulator of the

171 Factor H (FH) is one of the most important regulatory pr

172 The plasma complement regulator factor H (FH) is thought to be the main regulator that p

173 The complement factor H (FH) mutation R1210C, which was described in as

174 Numerous complement factor H (FH) mutations predispose patients to atypical

175 his because it recruits complement regulator factor H (FH) onto the bacterial surface to evade comple

176 binding of the alternative pathway regulator factor H (fH) to PorB of some strains.

177 ty by binding the human complement regulator factor H (fH) with high affinity, is also a key antigen

178 s complement-mediated destruction by binding factor H (FH), a host-derived negative regulator of comp

179 sms, mutations, and autoantibodies affecting factor H (FH), a major regulator of the alternative comp

180 P5 expression was required for NT127 to bind factor H (fH), an important inhibitor of alternative pat

181 The serum proteins factor H (FH), consisting of 20 complement control prote

182 Serum concentrations of factor H (FH), factor I (FI), C9, and C3 were measured,

183 their interaction with the control proteins factor H (FH), membrane cofactor protein (MCP; CD46), an

184 structurally related to complement inhibitor factor H (FH), the initial assumption was that the FHRs

185 ate complement system, many microbes capture factor H (FH), the key soluble complement-regulating pro

186 enzae lipoprotein having the ability to bind factor H (FH), the major regulator of the alternative pa

187 e physiological plasma complement regulator, factor H (FH), we studied the effect of Ecb on the compl

188 One such host regulator is factor H (FH), which acts as a negative regulator of com

189 The 155-kDa plasma glycoprotein factor H (FH), which consists of 20 complement control p

190 binds the complement downregulating protein, factor H (fH), which enables the organism to evade host

191 Autoantibodies targeting factor H (FH), which is a main alternative complement pa

192 catalyzed by factor I (FI) and its cofactor, factor H (FH), with or without the participation of huma

193 ructural insight on the complement regulator factor H (FH), yielding a novel complement-targeted ther

194 The meningococcal vaccine antigen, factor H (FH)-binding protein (FHbp), binds human comple

195 -specific ligand of the complement inhibitor factor H (FH).

196 nd factor (VWF) and the complement regulator factor H (FH).

197 converting the FHR-1 C terminus into that of factor H (FH).

198 d the inhibitory domain of the CAP regulator factor H (fH).

199 tein (MCP; CD46), but a normal regulation by factor H (FH).

200 t important regulators is the plasma protein factor H (FH).

201 pecific ligand for the complement regulator, factor H (fH).

202 s functional defects in complement regulator factor H (FH).

203 tions in the inhibitory complement regulator factor H (FH).

204 r of the AP is the plasma protein complement factor H (FH).

205 pathway activity is inhibited by complement factor H (FH).

206 ein contributes to immune evasion by binding factor H (FH).

207 We analyzed the efficiency of factor H (FH)6-7/Fc, a novel antibacterial immunotherape

208 ged with complement active serum depleted of Factor H, FHL-1, and CFHR1, demonstrating a protective r

209 ); between rs2669845 and Y402H in complement factor H for AMD (P = .04); and between rs2669845, rs285

210 esonance experiments showed high affinity of factor H for heparin and HS (K(D) values of 32 and 93 nm

211 Factor H forms large oligomers in >10 muM zinc.

212 Depletion of factor H from normal human serum renders R. conorii more

213 lase recruited C4BP and plasminogen, but not factor H, from human serum.

214 This suggests that factor H function is affected by local conditions within

215 platelets became complement susceptible when factor H function was blocked.

216 ted tissue inflammation by locally impairing factor H function, but it can also improve complement-me

217 s 358 kb region that contains the Complement Factor H gene cluster.

218 s have found variation within the complement factor H gene family links to host susceptibility to men

219 and the Y402H polymorphism in the complement factor H gene on chromosome 1q) and mortality.

220 Complement factor H genotype had no effect on the responses to pazo

221 Deficiency of complement factor H has long been associated with MPGN.

222 diseases, highlighting the critical role of factor H in AP regulation.

223 ed the expression of full-length recombinant factor H in moss (Physcomitrella patens).

224 gial immunodeposits, including properdin and factor H in the alternative pathway and mannan-binding l

225 the corresponding gene products compete with factor H in the regulation of the alternative pathway, i

226 beta peptide 1-42, C4b-binding protein, and factor H, in a CRP concentration- and ligand concentrati

227 Factor H is a circulating protein that regulates activat

228 Although factor H is a well known inhibitor of the alternative co

229 Factor H is an important complement regulator of the alt

230 ative pathway activation, but no therapeutic factor H is commercially available.

231 s 1q31.3, containing the gene for complement factor H (lead single nucleotide polymorphism: rs800292;

232 ently cleaved by factor I in the presence of factor H, leading to enhanced C3 fragment deposition on

233 carriers of rs800292 minor allele (Ile62 in factor H) led to decreased release of MMP-8 from neutrop

234 Carriers tended to have low serum Factor H levels, especially carriers of the splice varia

235 porter protein rickettsial OmpB (rOmpB) as a factor H ligand and further demonstrate that the rOmpB b

236 n complement regulators such as factor H and factor H-like protein 1 (FHL-1) for immune evasion.

237 ind to PTX3 and that PTX3-bound factor H and factor H-like protein 1 maintain their complement regula

238 In this study, we show that native factor H, factor H-like protein 1, and factor H-related protein 1

239 Inhibition of factor H-mediated cell surface complement regulation sig

240 ein stabilized the C3 convertase and reduced factor H-mediated convertase decay.

241 R4-C3bBb convertase is less sensitive to the factor H-mediated decay compared with the C3bBb converta

242 on causing aHUS, including 4 with complement factor H mutations.

243 A small fraction of C3G-DDD cases linked to factor H or C3 gene mutations as well as autoantibodies

244 however, neither OMP was found to bind human factor H or to be required for enhancing serum resistanc

245 idase 1) (P =2.7 x 10(-72)), CFH (complement factor H) (P =2.3 x 10(-47)), C2 (complement component 2

246 proximately 1 mg purified moss-derived human factor H per liter of initial P. patens culture after a

247 the human complement regulators factor I and factor H, promoting inactivation of C3b.

248 val of Crry/DAF-deficient platelets aided by factor H protection and compensatory thrombopoiesis demo

249 The complement factor H R1210C rare variant confers the strongest genet

250 The typical phenotype of the complement factor H R1210C rare variant is associated with extensiv

251 a novel heterozygous mutation in complement factor H (R83S) in addition to known risk polymorphisms

252 Furthermore, injection of moss-derived factor H reduced C3 deposition and increased serum C3 le

253 ying a genetic abnormality in the complement factor H related 1 gene (CFHR1) that originates by recur

254 g the genes encoding factor H (fH) and the 5 factor H related proteins have been described in associa

255 We demonstrate that complement factor-H related 3 (CFHR3) promotes immune activation by

256 Copy number variations in the complement factor H-related (CFHR) gene cluster on chromosome 1q32

257 ied a chromosomal deletion in the complement factor H-related (CFHR) gene cluster.

258 mmune renal disease deficient for complement factor H-related (CFHR) genes and autoantibody-positive

259 omplement factor H (CFH) and five complement factor H-related (CFHR) genes.

260 genomic rearrangements within the complement factor H-related (CFHR) locus.

261 The role of the complement factor H-related (FHR) proteins in homeostasis, pathogen

262 ated genomic mutation in the gene complement factor H-related 1 (CFHR1), which encodes FHR1.

263 hat a duplication within the gene complement factor H-related 1 (CFHR1; encoding FHR1) leads to the p

264 udies have shown that deletion of complement factor H-related genes 1 and 3 (CFHR3,1Delta) is associa

265 on studies identified deletion of complement factor H-related genes 1 and 3 as protective against the

266 Factor H-related protein (FHR) 1 is one of the five huma

267 ative factor H, factor H-like protein 1, and factor H-related protein 1 (CFHR1) bind to PTX3 and that

268 ociation of rs7517126 with plasma complement factor H-related protein 1 (CFHR1) level at p<1.46x10(-6

269 nous nitric oxide production) and complement factor H-related protein 2 (CFHR2, related to complement

270 Complement factor H-related proteins (FHRs) are strongly associated

271 locus is complex and biological roles of the factor H-related proteins, unlike factor H, are incomple

272 est decreased inhibition of C3 by complement factor H, resulting in increased activation of the alter

273 Interactions with complement factor H (rs1061170), age-related maculopathy susceptibi

274 This glycosylation-optimized factor H showed full in vitro complement regulatory acti

275 These genes include the sigma factor H (sigH) that was shown to be important for M. av

276 deliver the complement-regulatory region of factor H specifically to the site of inflammation provid

277 The role of complement factor H still needs to be better defined, but in light

278 Residues within short consensus repeat 20 of factor H that are relevant for PTX3 binding were identif

279 by the first five SCR domains of complement factor H that bind to complement C3b.

280 diseases affect the glycoprotein complement factor H, the main regulator of the alternative pathway

281 howed that annexin A2 reduces the binding of factor H to cell surfaces.

282 C3b, then C3b is inactivated by Factor I and Factor H to form the C3c and C3d fragments.

283 We used purified murine factor H to immunoprecipitate binding partners from mous

284 hat inducible proteins impair the ability of factor H to locally control the AP, thereby increasing A

285 nt of the complement downregulators C4BP and factor H to the pneumococcal cell wall and directly clea

286 allenged the current direct binding model of factor H to YadA and show that Y. enterocolitica YadA re

287 itors such as C4b-binding protein (C4BP) and factor H via pneumococcal surface protein C, thereby inh

288 his study, we show that M. catarrhalis binds factor H via the outer membrane protein OlpA.

289 stic of sub-RPE deposits, such as complement factor H, vitronectin, and amyloid beta, revealed that H

290 otein and positively with afamin, complement factor H, VLDL-associated apolipoproteins, and lipid sub

291 Binding of mutant C3 to factor H was normal, but mutant C3 was less efficiently

292 Factor H was present on the urinary side of renal tubula

293 L-6), interleukin-10 (IL-10), and complement factor H was unaffected.

294 ly by binding C4b binding protein (C4BP) and factor H, which are both complement inhibitors.

295 vating protein, factor B, and the inhibitor, factor H, which are opposite effects that complicate its

296 ptors, IgM natural antibodies and complement factor H, which bind, neutralize and/or facilitate their

297 In contrast, Factor H, which binds to the N-terminal part of mature P

298 so had decreased binding of human complement Factor H, which in previous studies increased the protec

299 he FHR3 binding sites on C3d are occupied by factor H, which lacks B cell-inhibitory functions.

300 y Susceptibility 2 rs10490924 and Complement Factor H Y402H (P for trend = 4.2x10(-7)).