ライフサイエンスコーパス: factor IX

1 ch TF-stimulated thrombin generation at 100% factor IX.

2 ves as an independent enzyme with respect to factor IX.

3 with inhibitory antibodies to Factor VIII or Factor IX.

4 it had normal affinity for the propeptide of factor IX.

5 eno-associated virus vector expressing human Factor IX.

6 icantly to the affinity of factor XIa toward factor IX.

7 calcium-stabilized form of the Gla domain of Factor IX.

8 mpared their behavior with that of wild-type factor IX.

9 y related to the circulatory levels of human factor IX.

10 sion of therapeutically beneficial levels of factor IX.

11 eno-associated viral (AAV) vector expressing factor IX.

12 e or in hemophilia B mice with inhibitors to factor IX.

13 that contributes to hemostasis by activating factor IX.

14 ed thrombin generation through activation of factor IX.

15 to lead to production of a truncated form of factor IX.

16 lid phase peptide synthesis and crystallized Factor IX-(1-47) in complex with Fab fragments of the 10

17 e overall structure of the Gla domain in the Factor IX-(1-47)-antibody complex at 2.2 A is similar to

18 IX-deficient plasma supplemented with mutant factor IX(a) proteins demonstrated resistance to DHG inh

19 ce to DHG inhibition of thrombin generation [factor IX(a) R233A > R170A > WT] that inversely correlat

20 nt adeno-associated virus-2 expressing human factor IX (AAV2-FIX), we detected 2 impediments to long-

21 h factor IX peptide bonds prior to releasing factor IX abeta.

22 n, exhibited similar potencies in inhibiting factor IX activation and the cleavage of a tripeptidic c

23 actor XI in a plasma-clotting assay and in a factor IX activation assay both in the presence and abse

24 specificity to FX activation as compared to factor IX activation by ETC.

25 irected inhibitors to study the mechanism of factor IX activation by factor XIa.

26 ited both factor XIa amidolytic activity and factor IX activation in a concentration-dependent manner

27 e competitive component of the inhibition of factor IX activation suggests that binding of factor IX

28 However, the inhibition of factor IX activation was best described by mixed-type in

29 g, prothrombin time, as well as factor X and factor IX activation.

30 In contrast, there was no inhibition of factor IX activation.

31 X are detectable throughout development, but factor IX activity is less than 1% and the mouse exhibit

32 IL-6, IL-10, d-dimer, antithrombin-III, and factor IX (adjusted HR = 1.27, p = 0.17).

33 However, factor IX alpha accumulated during activation by the fac

34 ese bonds sequentially, with accumulation of factor IX alpha, an intermediate cleaved after Arg145.

35 rter protein consisting of the gla domain of factor IX (amino acids 1-46) and residues 47-420 of prot

36 romote the delivery of the human coagulation factor IX and alpha-galactosidase genes into endogenous

37 e membrane binding site in the omega loop of Factor IX and blocks Factor IX function by inhibiting it

38 relationships of F7 and F10 with Factor VII, Factor IX and cholesterol levels.

39 We demonstrated that coagulation factor IX and complement component C4-binding protein ca

40 ing empty vector capsids, the utilization of factor IX and factor VIII modified transgenes to improve

41 On the other hand, two genes were found for factor IX and four for factor VII.

42 d flow cytometry to visualize the binding of factor IX and IXa to thrombin- or SFLLRN-activated plate

43 It also prevented activation of factor IX and prolonged human plasma and whole blood clo

44 l pharmacokinetic assessments of recombinant factor IX and rFIXFc.

45 factor XIa binds with comparable affinity to factors IX and IXabeta and that the interactions are dep

46 Plasma factors IX and IXabeta bind to factor XIa with K(d) valu

47 We examined binding of factors IX and IXabeta to factor XIa by surface plasmon

48 However, its specificity for human factors IX and X (FIX and FX) has limited its in vivo fu

49 Therapeutic expression of human clotting factors IX and X following adeno-associated viral vector

50 is also known to bind the natural substrates factors IX and X, thereby facilitating their assembly an

51 Only elevated levels of factors IX and XI were associated with increased risk of

52 n three blinded analyses of the factor VIII, factor IX, and ATM genes.

53 X chromosome that encodes blood coagulation factor IX, and is predicted to alter RNA splicing and to

54 , the interaction of FXIa with the substrate factor IX, and the binding of FXI to platelets.

55 ely the bone health of clotting factor VIII, factor IX, and Von Willebrand Factor knockout (FVIII(-/-

56 Conformation-specific anti-Factor IX antibodies are directed at the calcium-stabili

57 and suggests their potential importance for factor IX antibody development in humans with hemophilia

58 ypothesis that H-2 (and other) genes control factor IX antibody development in mice and suggests thei

59 (LOD scores of approximately 2.3-2.6) to the factor IX antibody response.

60 from patients with hemophilia A, and in anti-factor IX antibody-induced ("acquired") hemophilia B blo

61 Plasma factor IX antigen remained at around 9%, 13%, and 16% of

62 When an S2'-P2' interaction is involved (factor IX, antithrombin, APPI), beta-branching and incre

63 In immunohistochemical studies, factor IX appears on the endothelial surfaces of mouse a

64 that persistently high circulatory levels of factor IX are a risk factor not only for thrombosis and/

65 tor IX mRNA transcript and circulating human factor IX are detectable throughout development, but fac

66 to correlate known hemophilia B mutations of factor IX at Lys5 or Phe9 with impaired phosphatidylseri

67 Transgenic mice overexpressing human factor IX at persistently high levels died at much young

68 Brain-derived neurotrophic factor IX (BDNF IXabcd) was selected as a readout gene t

69 or IXa with the density of binding sites for factor IX being about half of that for factor IXa, consi

70 bitor of FVIIa/TF as assessed by recombinant factor IX (BeneFIX) activation assays.

71 onoclonal antibody was a potent inhibitor of factor IX binding to factor XIa (K(i) 34 nm) and activat

72 ibited by factor IX, consistent with loss of factor IX-binding exosites on the non-catalytic factor X

73 Thus, we have demonstrated that factor IX binds in vivo to endothelial cell-collagen IV

74 The results support a model in which factor IX binds initially to exosites on the factor XIa

75 bolic mixed-type inhibition, indicating that factor IX binds to free and S2366-bound factor XIa at ex

76 Recombinant factor IX bound to factor XIa with a K(d) of 107 nm, whe

77 The mouse expresses no mouse factor IX, but instead expresses a missense mutant human

78 d mouse strains were immunized against human factor IX by adenoviral gene transfer or serial injectio

79 mise, inhibition of factor XIa activation of factor IX by aprotinin (Ki 0.89 +/- 0.52 microM) was non

80 tes per HUVEC and promotes the activation of factor IX by cell bound FXIa.

81 troaniline (S-2366) and on the activation of factor IX by factor XIa have been investigated.

82 To test this, we examined cleavage of factor IX by four single active site factor XIa protease

83 repared the fully carboxylated Gla domain of Factor IX by solid phase peptide synthesis and crystalli

84 ce of exosite interactions in recognition of factor IX by the protease factor XIa.

85 ure in the detection of human blood clotting factor IX by voltammetry.

86 We then packaged the therapeutic factor IX cassette into haploid AAV2/8 1:3 capsids and i

87 We measured plasma canine factor IX (cFIX) concentrations at a therapeutic range f

88 on of 10% and 26% of normal levels of canine factor IX (cFIX) for more than a year.

89 will complement the FIXKO mice for studying factor IX circulating kinetics and gene therapy.

90 We found sustained therapeutic expression of factor IX coagulant activity after gene transfer in 10 p

91 Transgene-derived factor IX coagulant activity enabled the termination of

92 y weight in 10 men with hemophilia B who had factor IX coagulant activity of 2% or less of the normal

93 rticipants, with a mean (+/-SD) steady-state factor IX coagulant activity of 33.7+/-18.5% (range, 14

94 Vector-derived factor IX coagulant activity was sustained in all the pa

95 howed 7% wild-type activity that depended on factor IX coexpression, indicating a VKD protein effect

96 of leupeptin and aprotinin to the factor XIa-factor IX complex only approximately 10-fold lower than

97 lues, bleeding frequency, and consumption of factor IX concentrate were prospectively evaluated after

98 leeding episodes and the use of prophylactic factor IX concentrate.

99 ells/collagen IV plays a role in controlling factor IX concentration in the blood.

100 The serum factor IX concentrations, while remaining in the therape

101 i 38 +/- 14 microM) but was not inhibited by factor IX, consistent with loss of factor IX-binding exo

102 We injected recombinant factor IX containing mutations at residue 5 (K5A, K5R) i

103 n the first gene therapy success and achieve factor IX correction sufficient to prevent bleeding with

104 A (factor VIII deficiency) and hemophilia B (factor IX deficiency) have now been achieved in patients

105 hypercholesterolemia, primary oxalosis, and factor IX deficiency, among others, might be amenable to

106 e the hemophilias factor VIII deficiency and factor IX deficiency.

107 ining mutations at residue 5 (K5A, K5R) into factor IX-deficient mice and compared their behavior wit

108 t protein in the bloodstream of hemophiliac, factor IX-deficient mice.

109 Factor IX-deficient plasma supplemented with mutant fact

110 mented with 700 pM factor VIII or VIIIa, and factor IX-deficient plasma supplemented with plasma-deri

111 tissue factor (TF) addition to reconstituted factor IX-deficient plasma, factor IX R170A supported a

112 In previous work we transferred a human factor IX-encoding adeno-associated viral vector (AAV) i

113 h Ala in Gla-domainless forms of recombinant factor IX expressed in mammalian cells.

114 h a factor VII Gla domain (rFIX/VII-Gla) and factor IX expressed in the presence of warfarin (rFIX-de

115 > 3 years, with observation ongoing), robust Factor IX expression (circulating levels of 4%-14%) by m

116 AV8 vector resulted in long-term therapeutic factor IX expression associated with clinical improvemen

117 munofluorescent staining, we show persistent factor IX expression in injected muscle tissue.

118 ated virus (scAAV8) vector approach directed factor IX expression of up to 6% in a human trial, the a

119 associated virus expressing human factor IX, factor IX expression without the development of antibodi

120 two of six C57BL/6 and four of eight BALB/c factor IX (F-IX)-deficient mice survived for >7 days, ev

121 Therapeutic levels of human Factor IX (F.IX) are also produced at an approximately 1

122 erated mice with a range of mutations in the Factor IX (F.IX) gene; these more faithfully reflect the

123 While substantial levels of coagulation factor IX (F.IX) have been achieved using AAV serotype 2

124 ciated viral vector (rAAV) expressing canine Factor IX (F.IX) resulted in long-term expression of the

125 ciated virus (AAV)-mediated gene transfer of factor IX (F.IX) to the liver results in long-term expre

126 caused by absence of functional coagulation factor IX (F.IX).

127 emophilia B [deficiency in blood coagulation factor IX (F.IX)] by gene replacement therapy is hampere

128 In hemophilia B (coagulation factor IX [F.IX] deficiency), lack of endogenous F.IX an

129 epitopes derived from an ARF in coagulation factor IX (F9) cDNA can induce CTL reactivity, subsequen

130 , we target a promoterless human coagulation factor IX (F9) gene to the liver-expressed mouse albumin

131 istmas disease, arises from mutations in the factor IX (F9) gene.

132 n of adeno-associated virus expressing human factor IX, factor IX expression without the development

133 died were prothrombin, activated factor VII, factor IX, factor X, activated protein C, protein S, and

134 fficacy, and pharmacokinetics of recombinant factor IX Fc fusion protein (rFIXFc) in previously treat

135 A recombinant factor IX Fc fusion protein (rFIXFc) with a prolonged ha

136 ptide comprised of amino acids Gly4-Gln11 of factor IX (fIX(G4)(-)(Q11)) and constrained by an engine

137 IIa-dependent FXI activation, FXIa-dependent factor IX (FIX) activation, or platelet-derived polyP, r

138 evealed factor VIII (FVIII) activity of 16%, factor IX (FIX) activity of 74%, von Willebrand factor (

139 liorate bleeding risk and provide endogenous factor IX (FIX) activity/synthesis through a single trea

140 tion in 293- and BHK cell lines expressing r-factor IX (fIX) and endogenous carboxylase or overexpres

141 The complex of the serine protease factor IX (FIX) and its cofactor, factor VIII (FVIII), i

142 Factor IX (FIX) binds to collagen IV (Col4) in the suben

143 lood coagulation factor XIa (FXIa) activates factor IX (FIX) by cleaving the zymogen at Arg(145)-Ala(

144 induction of immune tolerance to coagulation factor IX (FIX) by direct intramuscular injection of ade

145 I (fXI) by thrombin and of the activation of factor IX (fIX) by fXIa.

146 induction of immune tolerance to coagulation factor IX (FIX) by hepatic adeno-associated viral (AAV)

147 ilia B is a bleeding disorder resulting from factor IX (FIX) deficiency that might be treated with ge

148 he long-term persistence (up to 10 years) of factor IX (FIX) expression in adeno-associated virus ser

149 l (AAV) vector-mediated gene transfer of the factor IX (FIX) gene in hemophilia B (HB) subjects with

150 ogs (n = 2) treated with liver-directed AAV2 factor IX (FIX) gene therapy did not have a single bleed

151 ugh the blinded analysis of mutations in the factor IX (FIX) genes of 88 hemophilia B (HB) patients a

152 ene therapy that induced immune tolerance to factor IX (FIX) in a hemophilia B (HB) dog with previous

153 sing a computational strategy that increased factor IX (FIX) levels 11- to 15-fold.

154 xpresses a codon-optimized hyperactive human factor IX (FIX) mutant (FIX Padua), it provides a >1 log

155 ing drug geneticin of 11 rationally selected factor IX (FIX) nonsense mutations, present in 70% (324/

156 combined deficiencies of Plg and coagulation factor IX (fIX) or XI (fXI) to determine the effects on

157 actor-like (EGF1) domain in factor X (FX) or factor IX (FIX) plays an important role in the factor VI

158 Current factor IX (FIX) products display a half-life (t(1/2)) of

159 cy and the risk of immunogenicity of a novel factor IX (FIX) R338L associated with approximately 8-fo

160 aused by the lack of factor VIII (FVIII) and factor IX (FIX) respectively, lead to insufficient throm

161 Binding of factor IX (FIX) to an exosite on the heavy chain of fact

162 hemophilia B requires frequent infusions of factor IX (FIX) to prophylax against bleeding episodes.

163 V) vector expressing a codon-optimized human factor IX (FIX) transgene (scAAV2/8-LP1-hFIXco) in a per

164 ed codon-usage optimized and hyperfunctional factor IX (FIX) transgenes carrying an R338L amino acid

165 a B mouse model with the expression of human factor IX (FIX) under control of the platelet-specific i

166 nction, we designed an optimized coagulation factor IX (FIX) variant and used multiple methods to com

167 BC-MVs), they document that RBC-MVs activate factor IX (FIX) via 2 distinct pathways: (1) the canonic

168 combinant fusion protein linking coagulation factor IX (FIX) with albumin (rIX-FP) which, along with

169 combinant fusion protein linking coagulation factor IX (FIX) with human albumin (rIX-FP) has been dev

170 rate (S-2366), the macromolecular substrate (factor IX (FIX)) and inhibitor PN2KPI.

171 lacement therapy with Factor VIII (FVIII) or Factor IX (FIX), either on demand to resolve bleeding, o

172 ncies in procoagulant factor VIII (FVIII) or factor IX (FIX), respectively.

173 re more resistant to thrombosis than fXI- or factor IX (fIX)-deficient mice, raising the possibility

174 hrough lipid nanoparticles (LNPs) to treat a Factor IX (FIX)-deficient mouse model of hemophilia B.

175 Analysis of factor IX (fIX)-expressing BHK cells indicated that slow

176 caused by absence of functional coagulation factor IX (FIX).

177 factors VIII and IX and anaphylaxis against factor IX (FIX).

178 on, the protease factor XIa (fXIa) activates factor IX (fIX).

179 3 x 10(12) vg/kg) encoding a hyperfunctional factor IX (FIX-Padua, arginine 338 to leucine) in FIX in

180 hodnius prolixus that binds with coagulation factors IX (fIX) and IXa (fIXa).

181 B (</=2 IU/dL [</=2%] endogenous coagulation factor IX [FIX] activity).

182 he enzymatic domain of activated coagulation factor IX (FIXa) is homologous to those of thrombin and

183 The assembly of the enzyme-activated factor IX (FIXa) with its cofactor, activated factor VII

184 o been implicated to interact with activated factor IX (FIXa).

185 ivity with the homologous protease activated factor IX (FIXa).

186 containing the propeptide and Gla domain of factor IX (FIXproGla41).

187 tain therapeutic levels of human coagulation Factor IX for more than six months in mice undergoing ex

188 ut instead expresses a missense mutant human factor IX from the mouse FIX promoter.

189 te in the omega loop of Factor IX and blocks Factor IX function by inhibiting its interaction with me

190 protein (F9CH) comprising the Gla domain of factor IX fused to the transmembrane and cytoplasmic reg

191 Oral delivery of coagulation factor IX fused with cholera toxin beta-subunit (with or

192 Residue K5 in factor IX gamma-carboxyglutamic acid (Gla) domain partic

193 ial of AAV2-mediated hepatic transfer of the Factor IX gene (F9) into hemophilia B subjects suggests

194 ult of missense mutations in the coagulation factor IX gene and defective circulating factor IX is de

195 deno-associated virus-mediated delivery of a Factor IX gene to skeletal muscle by direct intramuscula

196 have been achieved using AAV2 delivering the factor IX gene to the liver of adeno-associated virus (A

197 adeno-associated virus-based strategies for factor IX gene transfer in hemophilia B.

198 nciple, we resequenced a 20-bp region of the factor IX gene with a microarray of P*s.

199 at 2.2 A is similar to the structure of the Factor IX Gla domain in the presence of calcium ions as

200 ore, the calcium coordination network of the Factor IX Gla domain is different than in Gla domain str

201 similar to those found in the calcium-bound factor IX Gla domain, FIX(1-47)-Ca(2+).

202 d that the interactions are dependent on the factor IX Gla domain.

203 An anti-factor IX Gla monoclonal antibody was a potent inhibitor

204 Our results suggest that factor IX Gla-domain mediated binding to endothelial cel

205 iciencies of factor VIII (haemophilia A) and factor IX (haemophilia B) are well recognised, von Wille

206 ne tolerance to a secreted human coagulation factor IX (hF.IX) antigen by adeno-associated viral gene

207 we saw 10- to 20-fold higher levels of human factor IX (hF.IX) expression at a range of doses, and in

208 systematic study on human blood coagulation factor IX (hFIX) and anti-coagulant protein C (hPC) gene

209 determine the most robust human coagulation factor IX (hFIX) expression cassette in an adenovirus, w

210 y constructing a liver-restricted mini-human factor IX (hFIX) expression cassette that can be package

211 By targeting human factor IX (hFIX) expression to late-stage erythropoiesis

212 cassettes embedding a gfp gene or the human factor IX (hfIX) gene flanked by ITRs from AAV genotypes

213 ase from phage phiC31 to integrate the human Factor IX (hFIX) gene permanently into specific sites in

214 n of an adenoviral vector carrying the human factor IX (hFIX) transgene can induce immune tolerance o

215 with a needle to induce hemarthrosis; human factor IX (hFIX) was either injected through the needle

216 ng a retroviral vector (RV) expressing human factor IX (hFIX).

217 ecific expression of human blood coagulation factor IX (hFIX).

218 promoter-1 encoding either human coagulation factors IX (hFIX) or X (hFX) into Macaca fascicularis fe

219 e enables production of high levels of human factor IX in a murine model of hemophilia B.

220 T) with respect to their ability to activate factor IX in a plasma clotting assay, to hydrolyze the c

221 pothesis that the antibody response to human factor IX in mice is controlled by genetic factors, espe

222 esulted in therapeutic levels of circulating Factor IX in mice.

223 ith wild type or K5R had 79% of the injected factor IX in the liver after 2 minutes, whereas 17% rema

224 a model therapeutic gene, human coagulation Factor IX, in HEK293T cells.

225 endothelial surfaces of mouse arteries after factor IX injection and of human arteries from surgical

226 When we blocked the liver circulation before factor IX injection, 74% of K5A and 64% of K5R remained

227 During hemostasis, factor IX is activated by factors XIa or VIIa, by cleava

228 During hemostasis, factor IX is activated to factor IXabeta by factor VIIa

229 ong-term expression of therapeutic levels of factor IX is already a reality for a small number of pat

230 ion factor IX gene and defective circulating factor IX is detectable in most patients.

231 h gamma-carboxyglutamic acid (Gla) domain of factor IX is involved in phospholipid binding and is req

232 nhibition of factor XIa cleavage of S2366 by factor IX (Ki 224 +/- 32 nM) was characterized by hyperb

233 Factor IX knockout (FIX(-/-)) mice received a puncture o

234 The available mouse factor IX knockout models of hemophilia B (FIXKO mouse)

235 lder and had severe hemophilia B (endogenous factor IX level of </=2 IU per deciliter, or </=2% of no

236 igh-dose group, a consistent increase in the factor IX level to a mean (+/-SD) of 5.1+/-1.7% was obse

237 type 8 (AAV8) vector has been shown to raise factor IX levels for periods of up to 16 months.

238 ose association between elevated circulatory factor IX levels in mice with thrombosis as well as myoc

239 ium-stabilized Gla domain and interfere with Factor IX-membrane interaction.

240 binding stoichiometry of 1.9 +/- 0.4 mol of factor IX/mol of factor XIa (Kd = 70 +/- 40 nm).

241 We have created a human factor IX mouse model of hemophilia B (R333Q-hFIX mouse)

242 Mutant human factor IX mRNA transcript and circulating human factor I

243 (NFIA), nuclear factor IB (NFIB), or nuclear factor IX (NFIX) results in abnormal development of the

244 eered disulfide bond would assume the native factor IX omega-loop conformation in the absence of Ca(2

245 ient plasma supplemented with plasma-derived factor IX or 100 pM factor IXa, the EC(50) for DHG was s

246 sion of systemic transgene products (such as factor IX or erythropoietin) following in vivo administr

247 s, we injected mice with two different human factor IX or Escherichia coli lacZ-expressing AAV seroty

248 study may be an interactive site for either factor IX or factor X.

249 philia patients with autoantibodies to their factor IX or FVIII; however, its mechanism of action rem

250 l subpopulation (4-20%) of platelets binding factor IX or IXa with the density of binding sites for f

251 coagulation biomarkers (antithrombin-III and factor IX) (p < 0.05).

252 gineered capsid, liver-specific promoter and factor IX Padua (factor IX-R338L) transgene at a dose of

253 the two factor XIa active sites cleave both factor IX peptide bonds prior to releasing factor IX abe

254 interleukin-10), coagulation (antithrombin, factor IX, plasminogen activator inhibitor, d-dimer, thr

255 cing fXI with recombinant fXI that activates factor IX poorly, or fXI that is activated poorly by thr

256 Factor XIa also cleaves factor IX preferentially after Arg145, but little interm

257 For the propeptide of factor IX (proFIX18), FIXproGla41, and carboxylated FIXp

258 (d) values of these mutant enzymes for human factor IX propeptide varied from 0.5- to 287-fold when c

259 ally protected from chemical modification by factor IXs propeptide.

260 ically effective, like the recombinant human factor IX protein (rhFIX) that is the current standard o

261 V-based lentiviral vector encoding the human factor IX protein into the fetal circulation of immunoco

262 gene transfer or serial injections of human factor IX protein.

263 amma-carboxylated recombinant human clotting factor IX (r-hFIX), cell lines stably overexpressing r-h

264 In this work we have used recombinant human factor IX (r-hFIX)-producing baby hamster kidney (BHK) c

265 to reconstituted factor IX-deficient plasma, factor IX R170A supported a 2-fold increase in velocity

266 pe) and peak thrombin concentration, whereas factor IX R233A had a 4- to 10-fold reduction relative t

267 liver-specific promoter and factor IX Padua (factor IX-R338L) transgene at a dose of 5x10(11) vector

268 globulin (factor VIII), or Christmas factor (factor IX), Rapaport and colleagues demonstrated that th

269 An antibody that blocks fXIa activation of factor IX reduced thrombin generation; however, an antib

270 In hemophilia B mice, factor IX replacement reduced the average time to hemost

271 ncies in coagulation factor VIII (FVIII) and factor IX, respectively, resulting in deficient blood co

272 uated the safety and efficacy of recombinant factor IX (rFIX) in previously untreated patients (PUPs)

273 to post-translationally process recombinant factor IX (rFIX) limit hemophilia B therapy to <20% of t

274 As compared with recombinant factor IX, rFIXFc exhibited a prolonged terminal half-li

275 ing exogenous factor IX, the blood levels of factor IX that bind to endothelial cells/collagen IV inc

276 The plasma concentration of factor IX that binds to endothelial cells/collagen IV (r

277 ated virus vectors resulted in expression of Factor IX that is 28-fold that obtained using single-str

278 he mouse with EDTA after injecting exogenous factor IX, the blood levels of factor IX that bind to en

279 For factor IX, the odds ratio (OR) was 1.4 (95% confidence i

280 At 5% factor IX, the times to occlusion for factor IX wild-typ

281 ophilia B, but because the models produce no factor IX they fail to reproduce the dominant human phen

282 We studied associations of coagulation factors IX through XIII with risk of future VTE in 2 gen

283 in a dose-dependent increase in circulating factor IX to a level that was 1 to 6% of the normal valu

284 The binding of factor IX to cell membranes requires a structured N-term

285 actor IX activation suggests that binding of factor IX to factor XIa heavy chain affects the interact

286 The binding of Factor IX to membranes during blood coagulation is media

287 tes, transaminitis, significant reduction in factor IX transgene expression, and loss of transduced h

288 this issue of Blood, Finn et al have taken a factor IX variant with increased specific activity assoc

289 intranasal administration of an AAV2/5-CC10-factor IX vector resulted in secretion of functional rec

290 els prior to the administration of AAV human factor IX vectors.

291 mice injected with K5A, 59% of the injected factor IX was found in liver and 31% was found in plasma

292 8 vector for liver-directed gene transfer of factor IX was not impacted by preimmunization with the o

293 pecific activities of plasma and recombinant factor IX were comparable (200 and 150 units/mg), wherea

294 seem to be represented by a single gene, and factor IX, which is ordinarily a cofactor of factor VIII

295 At 5% factor IX, the times to occlusion for factor IX wild-type, R170A, and R233A were 15.7 minutes,

296 3A had a 4- to 10-fold reduction relative to factor IX wild-type.

297 to factor XIa with a K(d) of 107 nm, whereas factor IX with a factor VII Gla domain (rFIX/VII-Gla) an

298 ctor XIa and 1/2-FXIa activate the substrate factor IX, with similar kinetic parameters in purified a

299 To test whether factor IX within the joint space can protect joints from

300 activity of the vitamin K-dependent clotting factors IX, X, and prothrombin.