ライフサイエンスコーパス: factor VIII

1 ith platelet GPIbalpha and collagen and with factor VIII.

2 oimmune disease caused by an autoantibody to factor VIII.

3 ilia A is hampered by the short half-life of factor VIII.

4 the apparent membrane affinity for wild-type factor VIII.

5 des promoting platelet adhesion, VWF carries Factor VIII.

6 mpairment of mutants compared with wild-type factor VIII.

7 taining von Willebrand factor or recombinant factor VIII.

8 fied from baby hamster kidney cell-expressed factor VIII.

9 hibitors than those treated with recombinant factor VIII.

10 determinant in the clearance of coagulation factor VIII.

11 ent in patients with nonsevere hemophilia A (factor VIII 2-40 IU/dL).

12 the 125 patients treated with plasma-derived factor VIII (20 patients had high-titer inhibitors) and

13 of the 126 patients treated with recombinant factor VIII (30 patients had high-titer inhibitors).

14 ; FvW:ristocetin cofactor activity 44 IU/dL; factor VIII 99%; normal multimeric plasma vWF pattern) w

15 in this exosite-dependent interaction is the factor VIII a2 segment (residues 711-740) separating the

16 V5) vector encoding a B-domain-deleted human factor VIII (AAV5-hFVIII-SQ) in nine men with severe hem

17 Factor VIII activation profiles paralleled the results a

18 von Willebrand factor propeptide (VWFpp) or factor VIII activity ( FVIII: C) and VWF antigen (VWF:Ag

19 nd subsequently alone, as long as hemostatic factor VIII activity (FVIII : C) levels were maintained.

20 ment was performed for VWF antigen (VWF:Ag), factor VIII activity (FVIII:C), blood group, and age.

21 E-1 and phenotypic functions of LSEC such as factor VIII activity and AcLOL uptake in cocultured LSEC

22 Factor VIII activity declined at 4 and 6 hours (p < 0.05

23 sociated with the sustained normalization of factor VIII activity level over a period of 1 year in si

24 In the high-dose cohort, the factor VIII activity level was more than 5 IU per decili

25 Factor VIII activity levels remained at 3 IU or less per

26 von Willebrand factor propeptide (VWFpp) or factor VIII activity to VWF antigen.

27 nsecutive patients with severe hemophilia A (factor VIII activity, <0.01 IU per milliliter) who were

28 ment of bleeding requires agents that bypass factor VIII activity.

29 The development of neutralizing anti-factor VIII alloantibodies (inhibitors) in patients with

30 be a risk factor for the development of anti-factor VIII alloantibodies.

31 rval [CI], 18.4 to 35.2) with plasma-derived factor VIII and 44.5% (95% CI, 34.7 to 54.3) with recomb

32 It serves as a carrier for factor VIII and acts as a vascular damage sensor by attr

33 hrombin split products, D-dimer, P-selectin, factor VIII and C-reactive protein.

34 aining of the selected histologic slide with factor VIII and CD105 antigens by using Spearmen rank co

35 n rates of thrombin-catalyzed proteolysis of factor VIII and consequent activation, the acidic to Ala

36 Factor VIII and factor V share structural homology and b

37 severe hemophilia (n = 4899 men with severe factor VIII and IX deficiency).

38 ailable genomic sequence data on coagulation factor VIII and predictive models of molecular evolution

39 and soluble P-selectin and also for clotting factor VIII and the thrombin generation potential.

40 nous thrombosis risk via concurrently raised factor VIII and von Willebrand factor levels.

41 dominance effects only at the ABO locus for factor VIII and von Willebrand factor.

42 ng kidney function in controls, most notably factor VIII and von Willebrand factor.

43 o, we achieved long-term expression of human factors VIII and IX (hFVIII and hFIX) in mouse models of

44 pressed inhibitor formation directed against factors VIII and IX and anaphylaxis against factor IX (F

45 ciency or dysfunction of coagulation protein factors VIII and IX, respectively.

46 mas from hemophilia A or C patients who lack factors VIII and XI, which are mediators of the two prin

47 to cleave substrates, including fibrinogen, factor VIII, and PAR1.

48 Increase in fibrinogen, factor VIII, and von Willebrand factor and decrease in a

49 everal studies showed that neutralizing anti-factor VIII (anti-fVIII) antibodies (inhibitors) in pati

50 Using coagulation factor VIII as a model ligand, we now study the contribu

51 Recombinant factor VIII as well as bypassing agents and immune toler

52 The boy had hemophilia due to a factor VIII autoantibody and nephrotic syndrome.

53 ssociation of immediate height velocity with factor VIII (beta = -2.16, 95% CI: -4.62, 0.29) and von

54 n, indicate an influence of the C1 domain on factor VIII binding to factor X, and indicate that coope

55 Factor VIII binds to phosphatidylserine (PS)-containing

56 ody BO2C11 decreased the V(max) of wild-type factor VIII by 90% but decreased the activity of 3 mutan

57 ults identify a membrane-binding face of the factor VIII C1 domain, indicate an influence of the C1 d

58 We prepared 4 factor VIII C1 mutations localized to a hypothesized mem

59 e an x-ray crystallographic structure of the factor VIII C2 domain in complex with 2 antibodies that

60 The factor VIII C2 domain is a highly immunogenic domain, wh

61 recognizes epitopes on opposing faces of the factor VIII C2 domain.

62 al" types of antibody inhibitors against the factor VIII C2 domain.

63 ciency virus type 1 antigens and coagulation factor VIII captured on the cantilever in the presence o

64 ction of recombinant B-domain-deleted canine factor VIII (cFVIII-BDD) unexpectedly revealed superior

65 (VWF:Act), antigen (VWF:Ag), multimers, and factor VIII coagulant activity were virtually absent.

66 nts with hemophilia B, the large size of the factor VIII coding region has precluded improved outcome

67 philia A, and no previous treatment with any factor VIII concentrate or only minimal treatment with b

68 data that take exposure days to therapeutic factor VIII concentrates into account.

69 py with plasma-derived von Willebrand factor-factor VIII concentrates represents the safe mainstay of

70 llance studies for all of the new engineered factor VIII concentrates with prolonged half-lives that

71 n (DDAVP) and/or von Willebrand factor (VWF)/factor VIII concentrates.

72 Factor VIII consists of a heavy chain (A1A2B domains) an

73 Patients treated with plasma-derived factor VIII containing von Willebrand factor had a lower

74 s among patients treated with plasma-derived factor VIII containing von Willebrand factor or recombin

75 Murine models of combined TFPI and factor VIII deficiency were used to examine the impact o

76 Black patients with hemophilia A (factor VIII deficiency) are twice as likely as white pat

77 In factor VIII-deficient plasma supplemented with 700 pM fa

78 cluding synthesis and release of coagulation factor VIII, demonstrated that transplanted cells were f

79 Repeated infusions of recombinant Factor VIII did not rescue thrombosis in VWF(-/-) mice,

80 s of a model protein substrate, procoagulant factor VIII, did not correlate with that of PFR-MCA prio

81 an second-generation full-length recombinant factor VIII, effect estimates remained similar for all i

82 ith animal growth to 5 wk of age with stable factor VIII expression thereafter to >1 y of age.

83 Decline in factor VIII expression was not related to cell-mediated

84 f choices among several commercial brands of factor VIII extracted from human plasma or engineered fr

85 Combined deficiency of factor V and factor VIII (F5F8D) is a bleeding disorder caused by mut

86 Combined deficiency of factor V and factor VIII (F5F8D) is caused by mutations in one of 2 g

87 constitute an exosite-interactive region in factor VIII facilitating cleavages for procofactor activ

88 ype (WT), baby hamster kidney cell-expressed factor VIII, factor IXa, and phospholipid vesicles to de

89 In a mechanism distinct from factor VIII, factor V activation involves proteolytic re

90 A recombinant factor VIII-Fc fusion protein (rFVIIIFc) was constructed

91 Neutralizing antibodies (inhibitors) toward factor VIII form a severe complication in nonsevere hemo

92 We previously demonstrated that coagulation factor VIII (FVIII) accelerates proteolytic cleavage of

93 iological pH and ionic strength, coagulation factor VIII (FVIII) accelerates, by a factor of approxim

94 Further testing revealed factor VIII (FVIII) activity of 16%, factor IX (FIX) act

95 e development of alloantibodies that inhibit factor VIII (FVIII) activity, Hay and DiMichele compared

96 ially influence the therapeutic qualities of factor VIII (fVIII) administration.

97 B are caused by deficiencies in coagulation factor VIII (FVIII) and factor IX, respectively, resulti

98 brinogen, coagulation factor VII (FVII), and factor VIII (FVIII) and its carrier von Willebrand facto

99 we demonstrate that the interaction between factor VIII (FVIII) and LRP1 occurs over an extended sur

100 Plasma factor VIII (FVIII) and von Willebrand factor (VWF) circ

101 Complex formation between coagulation factor VIII (FVIII) and von Willebrand factor (VWF) is o

102 ic response to vascular injury requires both factor VIII (FVIII) and von Willebrand factor (VWF).

103 investigations into the interaction between factor VIII (FVIII) and von Willebrand factor (VWF).

104 ence a higher incidence of neutralizing anti-factor VIII (FVIII) antibodies ("inhibitors") vis-a-vis

105 severe hemophilia A develop inhibitory anti-factor VIII (fVIII) antibodies (Abs).

106 Neutralizing anti-factor VIII (FVIII) antibodies that develop in patients

107 with hemophilia A is the development of anti-factor VIII (fVIII) antibodies.

108 Inhibitory antibodies to factor VIII (FVIII) are a major complication in the trea

109 The primary B-cell epitopes of factor VIII (fVIII) are in the A2 and C2 domains.

110 Frequent infusions of intravenous factor VIII (FVIII) are required to prevent bleeding ass

111 A long-acting factor VIII (FVIII) as a replacement therapy for hemophi

112 hestrate hemostatic processes, in particular factor VIII (FVIII) binding and stabilization in plasma,

113 The primary cellular source of factor VIII (FVIII) biosynthesis is controversial, with

114 e uptake and processing of blood coagulation factor VIII (FVIII) by antigen-presenting cells and the

115 s may avoid inhibitory antibody formation to factor VIII (FVIII) by taking advantage of immune immatu

116 Factor VIII (FVIII) consists of a heavy (A1A2B domains)

117 Factor VIII (FVIII) consists of a heavy chain (A1(a1)A2(

118 The factor VIII (FVIII) crystal structure suggests a possibl

119 Human and porcine coagulation factor VIII (fVIII) display a biosynthetic efficiency di

120 Although genetic induction of factor VIII (FVIII) expression in platelets can restore

121 illebrand factor (VWF) D'D3 domains protects factor VIII (FVIII) from rapid clearance.

122 wn to mediate clearance of blood coagulation factor VIII (FVIII) from the circulation.

123 Factor VIII (FVIII) functions as a cofactor for factor I

124 mophilia A is caused by mutations within the Factor VIII (FVIII) gene that lead to depleted protein p

125 Membrane-bound Factor VIII (FVIII) has a critical function in blood coa

126 The C1 domain of factor VIII (FVIII) has been implicated in binding to mu

127 III (rFVIII), comprised of covalently bonded factor VIII (FVIII) heavy and light chains.

128 risk factors for the initiation of the anti-factor VIII (FVIII) immune response seen in 25% to 30% o

129 f B-cell depletion on tolerance induction to factor VIII (FVIII) in a mouse model of hemophilia A.

130 in of von Willebrand factor (VWF) stabilizes factor VIII (FVIII) in the circulation and maintains it

131 f neutralizing antibodies (inhibitors) after factor VIII (FVIII) infusions is a serious complication

132 et al provide evidence that the high rate of factor VIII (FVIII) inhibitors seen in black hemophilia

133 ral cell type(s) that synthesize and release factor VIII (FVIII) into the circulation are still not k

134 The development of inhibitory antibodies to factor VIII (FVIII) is a major obstacle in using this cl

135 Recombinant canine B-domain deleted (BDD) factor VIII (FVIII) is predominantly expressed as a sing

136 ating hemophilia A patients with therapeutic factor VIII (FVIII) is that 20% to 30% of these patients

137 antibodies (inhibitors) against coagulation factor VIII (FVIII) is the most problematic and costly c

138 Replacement therapy with factor VIII (FVIII) is used in patients with hemophilia

139 ariants with von Willebrand factor (VWF) and factor VIII (FVIII) levels in 4468 AAs.

140 The development of anti-factor VIII (FVIII) neutralizing antibodies (inhibitors)

141 Thrombin-catalyzed activation of factor VIII (FVIII) occurs through proteolysis at three

142 ia is treated by IV replacement therapy with Factor VIII (FVIII) or Factor IX (FIX), either on demand

143 haracterized by deficiencies in procoagulant factor VIII (FVIII) or factor IX (FIX), respectively.

144 th or without inhibitory antibodies to human factor VIII (FVIII) or IX (FIX).

145 Previous studies have demonstrated that factor VIII (FVIII) or platelets alone increase cleavage

146 ecutive anti-CD3 treatments concomitant with factor VIII (FVIII) plasmid injection prevented the form

147 Gene transfer of a factor VIII (FVIII) plasmid into hemophilia A (HemA) mic

148 data after the infusion of a new recombinant factor VIII (FVIII) product that when fused with the Fc

149 Current factor VIII (FVIII) products display a half-life (t(1/2)

150 poses a challenge for optimal treatment with factor VIII (FVIII) products.

151 ent of neutralizing Abs to blood coagulation factor VIII (FVIII) provides a major complication in hem

152 Neutralizing antibodies against factor VIII (FVIII) remain the major complication in the

153 The cellular source of coagulation factor VIII (FVIII) remains controversial.

154 Although factor VIII (FVIII) replacement therapy can be lifesavin

155 lopment of neutralizing antibodies following factor VIII (FVIII) replacement therapy for hemophilia A

156 emophilia A, the most severe complication of factor VIII (FVIII) replacement therapy involves the for

157 acid substitution N1922S in the A3 domain of factor VIII (fVIII) results in moderate to severe hemoph

158 et al demonstrate that platelets expressing factor VIII (FVIII) shield FVIII from immune detection.

159 The liver is a major factor VIII (FVIII) synthesis site, and mesenchymal stem

160 sorder caused by a deficiency in coagulation factor VIII (fVIII) that affects 1 in 5,000 males.

161 ost feared treatment-related complication of factor VIII (fVIII) therapy.

162 Administration of human factor VIII (FVIII) to FVIII knockout hemophilia mice is

163 treatment, ranging from high-dose intensive factor VIII (FVIII) treatment to prophylactic treatment,

164 aluate the efficacy of a newly bioengineered factor VIII (fVIII) variant (efVIII)--containing a combi

165 s to describe the relationships of the PK of factor VIII (FVIII) with age and body weight by a popula

166 These approaches include factor VIII (FVIII) with extended half-life (eg, FVIII-F

167 Studies have correlated elevated plasma factor VIII (FVIII) with thrombosis; however, it is uncl

168 Neutralizing autoantibodies against factor VIII (FVIII), also called FVIII inhibitors, are t

169 Administration of recombinant factor VIII (FVIII), an important co-factor in blood clo

170 dylserine (PS) but express binding sites for factor VIII (fVIII), casting doubt on the role of expose

171 e protease factor IX (FIX) and its cofactor, factor VIII (FVIII), is crucial for propagation of the i

172 elial cells are a major endogenous source of Factor VIII (FVIII), lack of which causes the human cong

173 erves as the carrier protein for coagulation factor VIII (FVIII), protecting it from proteolytic degr

174 We have analyzed expression of coagulation factor VIII (FVIII), the protein deficient in hemophilia

175 ent of smaller expression cassettes encoding factor VIII (FVIII), which demonstrate improved biosynth

176 ron-22-inversion patients express the entire Factor VIII (FVIII)-amino-acid sequence intracellularly

177 lebrand factor (VWF) fragment containing the factor VIII (FVIII)-binding D'D3 region of VWF is suffic

178 een shown to increase the clot lysis time in factor VIII (fVIII)-deficient plasma by an activated thr

179 Previously, we delivered autologous factor VIII (FVIII)-expressing blood outgrowth endotheli

180 Factor VIII (FVIII)-neutralizing antibodies ("inhibitors

181 globulin (Ig) isotypes and IgG subclasses of factor VIII (FVIII)-specific antibodies are found in dif

182 Such engineered factor VIII (FVIII)-specific Tregs efficiently suppresse

183 disorder caused by deficiency of coagulation factor VIII (FVIII).

184 of inhibitor antibodies against coagulation factor VIII (fVIII).

185 ion of recombinant clotting factors, such as factor VIII (FVIII).

186 the distribution of plasma levels for FV and factor VIII (FVIII).

187 A (AHA) is caused by autoantibodies against factor VIII (FVIII).

188 izing antibodies (inhibitors) to replacement factor VIII (FVIII, either plasma derived or recombinant

189 d VWF antigen (VWF:Ag) and the ratio between factor VIII (FVIII:C) and VWF:Ag may be used to assess s

190 the combined deficiency of factor V (FV) and factor VIII (FVIII; F5F8D), suggesting an ER-to-Golgi ca

191 A2 domain rapidly dissociates from activated factor VIII (FVIIIa) resulting in a dampening of the act

192 ntribute to A2 domain retention in activated factor VIII (FVIIIa).

193 model in which exons 17 and 18 of the murine factor VIII gene (F8) are flanked by loxP sites, or flox

194 We sequenced the factor VIII gene (F8) in black patients with hemophilia

195 gest that bleeding phenotype correlates with factor VIII gene (F8) mutation type.

196 against vectors and by the large size of the factor VIII gene.

197 alternative treatment options to prolong the factor VIII half-life, and delineate the role of VWF and

198 an 87% higher incidence than plasma-derived factor VIII (hazard ratio, 1.87; 95% CI, 1.17 to 2.96).

199 plant species eliminated 105 (human clotting factor VIII heavy chain [FVIII HC]) and 59 (polio VIRAL

200 Human factor VIII (hFVIII) plasmid gene therapy in hemophilia

201 sociated virus (rAAV) vectors encoding human factor VIII (hFVIII) were systematically evaluated for h

202 otential complexities of the polyclonal anti-factor VIII immune response and further define the "clas

203 , and antibody responses against coagulation factor VIII in hemophilia A mice, even in animals previo

204 rminants of development of inhibitory Abs to factor VIII in people with hemophilia A indicate a compl

205 ematoma with midline shift, a single dose of factor VIII inhibitor bypassing activity (25 U/kg) was a

206 lation tests following the administration of factor VIII inhibitor bypassing activity and a follow-up

207 Factor VIII inhibitor bypassing activity may be a viable

208 randomized trial to assess the incidence of factor VIII inhibitors among patients treated with plasm

209 o explore the natural history of later-onset factor VIII inhibitors and to investigate other potentia

210 Patients with severe hemophilia A and factor VIII inhibitors are at increased risk for serious

211 Factor VIII inhibitors arise in patients with hemophilia

212 The age-adjusted incidence of new factor VIII inhibitors was analyzed in all United Kingdo

213 nts in patients with severe hemophilia A and factor VIII inhibitors.

214 on in an NHP model of hemophilia A with anti-factor VIII inhibitors.

215 ices are price and the risk of occurrence of factor VIII inhibitory alloantibodies.

216 Factor VIII is activated by thrombin through proteolysis

217 The procofactor, factor VIII, is activated by thrombin or factor Xa-catal

218 factor IX, which is ordinarily a cofactor of factor VIII, is not present.

219 egimens for patients with severe hemophilia (factor VIII/IX < 1 IU/dL) born between 1970 and 1994, us

220 Bleeding in hemophilia is the result of factor VIII/IX deficiency with corresponding reduced thr

221 g quantified proteins C and S, antithrombin, factors VIII/IX/XI, fibrinogen, lipoprotein(a), homocyst

222 alysis of hematopoietic stem cells (HSCs) in factor VIII knockout (FVIIIKO) mice revealed a novel reg

223 The average factor VIII level in this group was 99 IU/dL, suggesting

224 e-matched analysis controlling for age, sex, factor VIII level, inhibitor titer, and underlying etiol

225 Compared with eGFR >50th percentile, factor VIII levels (adjusted mean difference, 60 IU/dL f

226 otypic remission 1 with normalization of VWF/factor VIII levels and multimer pattern.

227 inhibitor development in mild hemophilia A (factor VIII levels between 5 and 40 U/dL) is larger than

228 tor tests with decreased in vivo recovery of factor VIII levels.

229 leukoreduced red blood cells and coagulation factor VIII manufactured from blood of United Kingdom do

230 lk fat globule epidermal growth factor (EGF)-factor VIII (MFG-E8) mediates the clearance of apoptotic

231 Milk fat globule-epidermal growth factor-factor VIII (MFG-E8), a membrane-associated secretory gl

232 e exosomes that contain milk fat globule EGF factor VIII (MFGE8), a protein required to opsonize apop

233 or capsids, the utilization of factor IX and factor VIII modified transgenes to improve secretion or

234 on (70%), defined as inhibitor undetectable, factor VIII more than 70 IU/dL and immunosuppression sto

235 sults obtained evaluating proteolysis of the factor VIII mutants by factor Xa revealed modest rate re

236 Factor VIII mutants were active on additional membrane s

237 Recombinant B-domainless factor VIII mutants, R1689H and R1689Q were prepared and

238 In breeding studies, Factor VIII null (F8(-/-)) did not rescue the embryonic

239 es in patients with inhibitory antibodies to Factor VIII or Factor IX.

240 iency of intrinsic blood coagulation pathway factor VIII or IX, pharmacological agents that inactivat

241 II-deficient plasma supplemented with 700 pM factor VIII or VIIIa, and factor IX-deficient plasma sup

242 Adjustment for factor VIII or von Willebrand factor attenuated these od

243 Inhibitory antibodies to factors VIII or IX represent a serious complication for

244 a bleeding disorder caused by deficiency in factors VIII or IX, the two components of the intrinsic

245 celerin (factor V), antihemophilic globulin (factor VIII), or Christmas factor (factor IX), Rapaport

246 n of a misfolding-prone human blood clotting factor VIII, or after partial hepatectomy.

247 ich cannot bind platelets, blood coagulation factor VIII, or collagen, causing VWD through dominant-n

248 on Willebrand factor (p = 4.7 x 10(-57)) and factor VIII (p = 1.2 x 10(-36)).

249 in, fibrinogen, total homocysteine, D-dimer, factor VIII, plasmin-antiplasmin complex, and inflammati

250 We assessed von Willebrand factor (VWF), factor VIII, platelet activation and aggregation, platel

251 ung angiogenesis was assessed by quantifying factor VIII-positive microvessels and levels of von Will

252 ophilia A, it is unclear whether the type of factor VIII product administered and switching among pro

253 Recombinant and plasma-derived factor VIII products conferred similar risks of inhibito

254 n the analysis was restricted to recombinant factor VIII products other than second-generation full-l

255 t only two (H1 and H2) match the recombinant factor VIII products used clinically.

256 using synthetic peptides from regions of the Factor VIII protein where ns-SNPs occur and showed that

257 of neutralizing anti-drug-antibodies to the Factor VIII protein-therapeutic is currently the most si

258 nectin and milk-fat globule epidermal growth factor VIII protein.

259 found in all racial groups and are the only factor VIII proteins found in the white population to da

260 white patients to produce inhibitors against factor VIII proteins given as replacement therapy.

261 There are six wild-type factor VIII proteins, designated H1 through H6, but only

262 development of antibodies to capsid or human factor VIII proteins.

263 /d) and low-dose (LD; 50 IU/kg 3 times/week) factor VIII regimens in 115 "good-risk," severe high-tit

264 while differentiated EC clusters (expressing factor VIII related antigen (RA)) increased by 25% (p <

265 Rapid immunohistochemistry using factor VIII-related antibodies was performed on sections

266 he expression of a specific vascular marker, factor VIII-related antigen (FVIII-RAg), and several cel

267 al measurements of vessel density, and found factor VIII-related antigen levels to correlate with the

268 riations in vessel density by measurement of factor VIII-related antigen, we found KLK6 protein and m

269 the Bristol (UK) samples, by measurement of factor VIII-related antigen, which we showed to correlat

270 reby inhibitory antibodies develop following factor VIII replacement therapy for congenital hemophili

271 preliminary results suggest that mismatched factor VIII replacement therapy may be a risk factor for

272 profiles, and leads to a return to a normal factor VIII response in approximately 60% of patients.

273 ially administered together with recombinant factor VIII (rFVIII) and subsequently alone, as long as

274 The effect of recombinant factor VIII (rFVIII) brand on inhibitor development was

275 d phase 3 multicenter study of a recombinant factor VIII (rFVIII) product fused with the Fc fragment

276 Six recombinant factor VIII (rFVIII) products have been marketed worldwi

277 sk of inhibitor development with recombinant factor VIII (rFVIII) than plasma-derived concentrates (p

278 ) combined at a fixed ratio with recombinant factor VIII (rFVIII) were investigated in 32 subjects wi

279 in is a novel B-domain-truncated recombinant factor VIII (rFVIII), comprised of covalently bonded fac

280 l-length complementary DNA sequence of human factor VIII), second-generation full-length products wer

281 f anti-C2 inhibitory antibodies that bind to factor VIII simultaneously was investigated by x-ray cry

282 4.5% (95% CI, 34.7 to 54.3) with recombinant factor VIII; the cumulative incidence of high-titer inhi

283 ; falls in thrombin generation, Factor V and Factor VIII to 52%, 19% and 17% normal respectively; fur

284 mune tolerance induction with large doses of factor VIII to eradicate inhibitors.

285 Patients with hemophilia A rely on exogenous factor VIII to prevent bleeding in joints, soft tissue,

286 Plasma factor VIII to VWF antigen (VWF:Ag) ratios were signific

287 We hypothesized that mismatched factor VIII transfusions contribute to the high incidenc

288 he study to 1 event after gene transfer, and factor VIII use for participant-reported bleeding ceased

289 on of hemostasis and a profound reduction in factor VIII use in all seven participants.

290 e sites into the disulfide bond-cross-linked factor VIII variant.

291 tested (interleukin-6, d-dimer, coagulation factor VIII, von Willebrand factor, and homocysteine).

292 The patient received factor VIII/vWF concentrate both during and after surger

293 dependent adenoviral vector expressing human factor VIII was administered i.v. to neonatal hemophilia

294 ary end point of all inhibitors, recombinant factor VIII was associated with an 87% higher incidence

295 residues to thrombin-catalyzed activation of factor VIII were assessed following mutagenesis of acidi

296 No neutralizing antibodies to factor VIII were detected.

297 ously characterized an engineered variant of factor VIII which contains a disulfide bond between the

298 to induce clotting and activate factor V and factor VIII with rates similar to the plasma-derived mol

299 , we hypothesize that storage of coagulation Factor VIII within platelets may provide a locally induc

300 as operational tolerance was established to factor VIII without development of inhibitors; however,