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 sion of therapeutically beneficial levels of factor IX.

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

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

11 ity against restricted functional domains of factor IX.

12 dues (Gla domain) and the protease domain of factor IX.

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

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

15 that contributes to hemostasis by activating factor IX.

16 ed thrombin generation through activation of factor IX.

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

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

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

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

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

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

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

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

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

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

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

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

29 The catalytic efficiency of factor IX activation is similar to wild-type protein, ho

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

47 l pharmacokinetic assessments of recombinant factor IX and rFIXFc.

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

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

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

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

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

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

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

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

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 one metal binding site as compared to two in Factor IX binding protein and Factor IX/X binding protei

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

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

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

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

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

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

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

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

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

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

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

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

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

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

86 ions in circulating levels of factor VIII or factor IX can prevent most of the mortality and much of

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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 s zymogen factor IX, which blocks the shared factor IX/factor IXa binding site, the substrate, factor

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 sue factor (FVIIa*TF) complex activates both factor IX (FIX) and factor X (FX).

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

176 3 x 10(12) vg/kg) encoding a hyperfunctional factor IX (FIX-Padua, arginine 338 to leucine) in FIX in

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

248 Human plasma-derived factor IX (pdFIX) concentrates are routinely used to tre

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

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

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

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

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

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

255 ally protected from chemical modification by factor IXs propeptide.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

270 the safety and efficacy of recombinant human factor IX (rFIX) were evaluated.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

289 8 vector for liver-directed gene transfer of factor IX was not impacted by preimmunization with the o

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

291 In the presence of excess zymogen factor IX, which blocks the shared factor IX/factor IXa

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

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

294 3A had a 4- to 10-fold reduction relative to factor IX wild-type.

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

296 Factor XI-R226 activates factor IX with a Michaelis-Menten constant (K(m)) about

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

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

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

300 ared to two in Factor IX binding protein and Factor IX/X binding protein and none in flavocetin.