ライフサイエンスコーパス: FHF

1 FHF (fibroblast growth factor homologous factor) variant

2 FHF binding to IB2 facilitates recruitment of the MAPK p

3 FHF can be modeled in mice by administration of azoxymet

4 FHF dysfunction has been linked to neurological disorder

5 FHF has been reported in three families-the original Iri

6 FHF is a formidable clinical problem associated with a h

7 FHF is usually accompanied by massive hepatocellular dea

8 FHF rat livers exhibited reduced amino acid uptake, a sw

9 FHF-induced recruitment of p38delta to IB2 is accompanie

10 FHF/IB2 interaction is highly specific, as FHFs do not b

11 n pressure (CPP) before and during OLT in 12 FHF patients undergoing transplantation.

12 complex (t-Bu-bpy)Pd(IV)(p-FC(6)H(4))(F)(2)(FHF) (t-Bu-bpy = 4,4'-di-tert-butyl-2,2'-bipyridine).

13 We found that the AP-4-FHF interaction is mediated by direct binding of the AP-

14 Additionally, 42 FHF rats were killed in batches of six rats each 2, 6, 1

15 Two of 50 FHF (4%) and 10 of 104 control patients (10%) had EBV DN

16 Three of 50 FHF (6%) and 14 of 104 control patients (13%) were posit

17 ould be considered more frequently for adult FHF patients.

18 All FHF rats became comatose by 24 hours postoperatively.

19 imary cardiac Nav channel Nav1.5) that alter FHF binding result in human cardiovascular disease.

20 V DNA was observed in 3 of 10 North American FHF patients (30%) and 3 of 59 controls (5%) without ser

21 multipoint linkage analyses indicate that an FHF gene is likely to be located in an 8-cM interval bet

22 can rescue both ventricular SHF addition and FHF integrity.

23 rain is serine/threonine-phosphorylated, and FHF can serve as a substrate for p38delta in vitro.

24 , which compromises both SHF recruitment and FHF ventricular integrity.

25 hydrogen bond strength in bifluoride anion (FHF-).

26 erstanding the mechanism of HEV-1-associated FHF.

27 case a bifluoride complex Tp'Rh(PMe3)(C5NF4)(FHF) was crystallized.

28 aluates reported canonical and non-canonical FHF functions.

29 In transiently transfected 293 cells FHF-1 accumulates in the nucleus and is not secreted.

30 The majority of cryptogenic FHF cases cannot be attributed to infection with herpes

31 , and 4379 recipients underwent DCD-FHF, DBD-FHF and DCD-non-FHF, respectively.

32 t survival in DCD-FHF were comparable to DBD-FHF (67.9 vs. 77.6%, p = .63; 57.8% vs. 73.2%, p = .27)

33 raft survival in DCD-FHF was inferior to DBD-FHF (72.9% vs. 83.8%, p = .002), but comparable to DCD-n

34 nt survival in DCD-FHF are comparable to DBD-FHF and DCD-non-FHF.

35 m graft survival and patient survival in DCD-FHF are comparable to DBD-FHF and DCD-non-FHF.

36 Graft and patient survival in DCD-FHF improved over the study period.

37 One-year graft survival in DCD-FHF was inferior to DBD-FHF (72.9% vs. 83.8%, p = .002),

38 However, 3- and 5-year graft survival in DCD-FHF were comparable to DBD-FHF (67.9 vs. 77.6%, p = .63;

39 117, 3437, and 4379 recipients underwent DCD-FHF, DBD-FHF and DCD-non-FHF, respectively.

40 d biochemical approaches show that different FHF complexes associate with distinct motile cargos.

41 The mutations also disabled FHF modulation of voltage-dependent fast inactivation of

42 han hepatocytes in fVIII biosynthesis during FHF.

43 l be useful in characterizing changes during FHF, and in elucidating the effects of nutritional suppl

44 Each FHF is expressed in the developing and adult nervous sys

45 d 71% amino acid sequence identity, but each FHF shows less than 30% identity when compared with othe

46 fibroblast growth factor homologous factor (FHF) binding to the C-terminus of sodium channels not on

47 fibroblast growth factor homologous factor (FHF) proteins to delay Nav inactivation, distal axonal N

48 HF2B, a member of the FGF homologous factor (FHF) subfamily, as an interacting partner of Na(v)1.6.

49 fibroblast growth factor homologous factors (FHF).

50 n-A, non-B, non-C fulminant hepatic failure (FHF) (3 patients), graft-versus-host disease (4 patients

51 During human fulminant hepatic failure (FHF) circulating levels of most hemostatic proteins fall

52 for patients with fulminant hepatic failure (FHF) continues to be unmet.

53 Patients with fulminant hepatic failure (FHF) die with brain edema, exhibiting an increased cereb

54 ale who developed fulminant hepatic failure (FHF) during the second trimester of pregnancy and underw

55 Fulminant hepatic failure (FHF) in humans produces a bleeding diathesis due in larg

56 r mass, otherwise fulminant hepatic failure (FHF) may arise.

57 Patients with fulminant hepatic failure (FHF) often die awaiting liver transplantation.

58 on patients with fulminant hepatic failure (FHF) or chronic liver disease (cirrhosis) was investigat

59 l animal model of fulminant hepatic failure (FHF) resembling the clinical condition is needed.

60 d transplants for fulminant hepatic failure (FHF) were stratified separately from those having an ele

61 nce of developing fulminant hepatic failure (FHF) with significant mortality.

62 hways affected by fulminant hepatic failure (FHF) would help develop nutritional support and other no

63 ive treatment for fulminant hepatic failure (FHF), but its use is limited because of organ donor shor

64 ive treatment for fulminant hepatic failure (FHF), but postOLT mortality is higher for patients with

65 n-A, non-B (NANB) fulminant hepatic failure (FHF), but the frequency of infection with these agents h

66 ere patients with fulminant hepatic failure (FHF), in group 2 (n = 3) were patients with primary nonf

67 ntation (OLT) for fulminant hepatic failure (FHF), some patients develop cerebral injury secondary to

68 tresia (EHBA) and fulminant hepatic failure (FHF).

69 TV in cryptogenic fulminant hepatic failure (FHF).

70 in patients with fulminant hepatic failure (FHF).

71 all children with fulminant hepatic failure (FHF).

72 but rarely cause fulminant hepatic failure (FHF).

73 ransplantation in fulminant hepatic failure (FHF).

74 unknown, and only familial Hibernian fever (FHF) has been described as a distinct clinical entity.

75 erized of which is familial Hibernian fever (FHF).

76 ication of the fused toes homolog-Hook-FHIP (FHF) complex as a novel AP-4 accessory factor.

77 in the previously discovered FTS/Hook/FHIP (FHF) complex, which contains, besides FHIP and Hook prot

78 The FTS-Hook-FHIP ('FHF') cargo adaptor complex links dynein to cargo in hum

79 ook-FTS and Hook-interacting protein (FHIP) (FHF) complex, which interacts with the minus-end-directe

80 ogenitors, the first and second heart field (FHF and SHF).

81 stinct sets of first and second heart field (FHF and SHF, respectively) progenitors.

82 ardium) develops from the first heart field (FHF) and expands by adding second heart field (SHF) cell

83 ardiac progenitors of the first heart field (FHF) do not require TBX1 and segregate precociously from

84 rdiomyocytes derived from first heart field (FHF) progenitors assemble the linear heart tube.

85 helial transition, with a first heart field (FHF) ridge apposing a motile juxta-cardiac field (JCF).

86 s), and second, a loss of first heart field (FHF) ventricular cardiomyocytes due to disrupted cell po

87 populations, first and second heart fields (FHF, SHF), sequentially contribute to longitudinal subdi

88 liver transplant (OLT) and chemotherapy for FHF secondary to PHL.

89 rates that LT is the treatment of choice for FHF and results in durable survival.

90 undertaken of all patients undergoing LT for FHF at a single transplant center.

91 Between 1984 and 2008, all LT for FHF performed in recipients less than or equal to 18 yea

92 ne of the largest published series on LT for FHF, demonstrates a long-term survival of nearly 70% and

93 examine predictors of survival after LT for FHF.

94 edian age 20.2 years) required urgent LT for FHF.

95 e 3 patients developing AA following OLT for FHF achieved hematologic recovery 21 and 92 days after d

96 The 1,457 patients who underwent OLT for FHF in the United States between 1988 and 2003 were enro

97 rge cohort of patients who underwent OLT for FHF was evaluated to develop and validate a system usefu

98 ct the probability of survival after OLT for FHF.

99 4 days) in the 3 patients undergoing OLT for FHF; in contrast, AA developed in the other 9 patients a

100 ical for devising therapeutic strategies for FHF.

101 The best understood 'canonical' targets for FHF action are voltage-gated sodium channels, and recent

102 and other nonsurgical medical therapies for FHF.

103 consecutive adult patients transplanted for FHF in an attempt to determine the extent of the histolo

104 reat benefit for the patients suffering from FHF.

105 Furthermore, FHF inhibited intrahepatic aspartate synthesis, which re

106 7 degrees C shortened survival time by half, FHF rats were not warmed during the postinduction period

107 of the homology core domain of all 10 human FHF isoforms.

108 tivity, contrary to the observation in human FHF.

109 taminophen overdose, a common cause of human FHF.

110 with a cyclosporine-based regimen (n=23) in FHF.

111 selective to the brain, may be of benefit in FHF.

112 onfirmation of the roles of these changes in FHF is lacking.

113 and biochemical features seen clinically in FHF, including severely impaired ability of the residual

114 n FHF to donation after brain death (DBD) in FHF and DCD in non-FHF over a 15-year period.

115 Consideration of DCD in FHF could help expand the donor pool in this subset of c

116 ts (SRTR), we compared outcomes after DCD in FHF to donation after brain death (DBD) in FHF and DCD i

117 However, in FHF, c-kit mRNA levels were elevated in 3 of 6 specimens

118 map of metabolic alterations in the liver in FHF.

119 ed by loss- or gain-of-function mutations in FHF genes.

120 est c-kit staining, however, was observed in FHF, in which, in addition to the cells in the portal tr

121 We conclude that RI in FHF and RI requiring dialysis or liver-kidney transplant

122 predictors of graft and patient survival in FHF, while DCD status was only predictive of graft survi

123 These data suggest that in FHF patients who develop intracranial hypertension befor

124 The effect of an individual FHF on a specific VGSC varies greatly depending upon the

125 varies greatly depending upon the individual FHF isoform.

126 To induce FHF, rats were fasted for 36 hours, during which they re

127 ytic sites of fVIII biosynthesis by inducing FHF in mice using acetaminophen overdose, a common cause

128 is the treatment of choice for irreversible FHF, few investigations have examined pretransplant vari

129 lored fluorescent reporter system to isolate FHF and SHF progenitors from developing mouse embryos an

130 The approximately 500-kDa FHF complex contained all three Hook proteins, and small

131 inactivation, distal axonal Navs show little FHF association or FHF requirement for high-frequency tr

132 tatic factors in azoxymethane-induced murine FHF.

133 To examine this issue, we studied 50 NANB FHF patients and 104 liver transplant recipients from No

134 hepatitis B sequences in patients with NANB FHF may simply reflect geographic differences.

135 In contrast, all 9 non-FHF patients developing AA after OLT died, 5 due to infe

136 = .63; 57.8% vs. 73.2%, p = .27) and DCD-non-FHF (67.9% vs. 72.9%, p = .44; 57.8% vs. 66.6%, p = .06)

137 83.8%, p = .002), but comparable to DCD-non-FHF (72.9% vs. 82.7%, p = .23).

138 ients underwent DCD-FHF, DBD-FHF and DCD-non-FHF, respectively.

139 CD-FHF are comparable to DBD-FHF and DCD-non-FHF.

140 fter brain death (DBD) in FHF and DCD in non-FHF over a 15-year period.

141 However, in the non-FHF patient, AA occurs in older individuals later in the

142 16 patients transplanted for other causes of FHF (11 paracetamol overdose, 2 idiosyncratic drug react

143 , all mild) transplanted for other causes of FHF.

144 nsistently displayed signs characteristic of FHF, including elevated plasma aminotransferase activity

145 nity is narrow for the dramatic condition of FHF, wide acceptance of this procedure will be of great

146 in teleosts, the primordial contributions of FHF and SHF to heart structure and function remain incom

147 considered in the differential diagnosis of FHF without clear etiology because of the potential for

148 system that allows for the identification of FHF- progenitors and their descendants including left ve

149 ntification of a novel, conserved isoform of FHF-2 in chickens and mammals.

150 the characterization of multiple isoforms of FHF-1, -2, -3, and -4 which are generated through the us

151 Knockdown of FHF subunits resulted in dispersal of AP-4 and ATG9A fro

152 rity to FGFs [3, 6, 7], but the mechanism of FHF action has not been reported.

153 developed and characterized a novel model of FHF in rats that has a number of physiological and bioch

154 pplemented by other tissues in this model of FHF.

155 cRNA-seq analysis revealed a predominance of FHF differentiation using the small molecule Wnt-based 2

156 The faster inactivation rate of FHF-free Navs together with very low axonal leak conduct

157 most patients succumbing to the sequelae of FHF before the correct diagnosis is made.

158 In the setting of FHF, it affects young males in the early posttransplanta

159 l domains in the Hook1 and Hook2 subunits of FHF.

160 Moreover, we identified the N-terminus of FHF as the critical molecule responsible for A-type FHFs

161 uld offer major benefits in the treatment of FHF.

162 ly creating new avenues for the treatment of FHF.

163 be beneficial in prevention and treatment of FHF.

164 l axonal Navs show little FHF association or FHF requirement for high-frequency transmission, velocit

165 We report that during an initial phase, FHF precursors differentiate rapidly to form a cardiac c

166 nd MELD score may be useful for prioritizing FHF-NA candidates.

167 Here we report bifluoride salts (Q(+)[FHF](-), where Q(+) represents a wide range of cations)

168 that loss of tbx5a and pitx2 affect relative FHF versus SHF contributions to the heart.

169 five patients (61%) grafted for seronegative FHF had fibrosis (13 mild, 9 moderate, and 3 severe) in

170 omplications, six patients with seronegative FHF required retransplantation (2 = chronic rejection; 1

171 mapped by two different groups: one studying FHF, the other studying a similar dominantly inherited s

172 Thus, targeting FHF modulation of Nav1.8/Nav1.9 may serve as a promising

173 We show here that FHF-1 is associated with the MAP kinase (MAPK) scaffold

174 We predict that FHF loss-of-function mutations would adversely affect cu

175 The FHF particle only blocks sodium channels from the open s

176 The FHF patients experienced more episodes of acute rejectio

177 Among the FHF patients, tacrolimus reduced the actuarial incidence

178 e identified a new multiprotein complex, the FHF complex, containing FTS, members of the microtubule-

179 ineage tracing and live imaging, we find the FHF and SHF are subdivided into distinct pools of progen

180 In other eukaryotes, HOOK homologs form the FHF complex with FTS and FHIP to activate dynein-mediate

181 delineate the functional contribution of the FHF and SHF to the zebrafish heart using the cis-regulat

182 with the previously demonstrated role of the FHF complex in coupling organelles to the microtubule (M

183 er with the evolutionary conservation of the FHF isoforms among human, mouse, and chicken, these data

184 Members of the FHF subfamily share approximately 70% sequence identity,

185 lternative splicing of the first exon of the FHF-2 gene and is predicted to encode a polypeptide with

186 fever, as a prelude to identification of the FHF-susceptibility gene.

187 y incorporated in posterior locations of the FHF.

188 nts we identified a conserved surface on the FHF core domain that mediates channel binding in vitro a

189 Anchored along the ridge, the FHF epithelium rotates the JCF forward to form the initi

190 We show the FHF complex is partially conserved in T. gondii, consist

191 ubdivisions of the heart tube (HT), with the FHF contributing the left ventricle and part of the atri

192 s is the first case, to be compared with the FHF(-) anion, of a neutral species with a single symmetr

193 Furthermore, we tested the effect of these FHF-associated mutations on genotype 3 HEV (HEV-3) repli

194 Additionally, we demonstrated that these FHF-associated mutations do not appear to alter their se

195 otype 1 HEV (HEV-1) are reportedly linked to FHF clinical cases, but experimental confirmation of the

196 cardial drl reporter expression restricts to FHF descendants.

197 atic failure without acetaminophen toxicity (FHF-NA, n = 312) had the poorest survival probability wh

198 to activate FGFRs, suggesting that these two FHF residues contribute to the inability of FHFs to acti

199 thy based on altered Nav1.5 association with FHF proteins.

200 Patients who were diagnosed with FHF and admitted to the University of Chicago were eligi

201 ma in experimental models and in humans with FHF, an effect associated with normalization of CBF.

202 100% of 21 HEV-1 variants from patients with FHF in Bangladesh.

203 ostOLT mortality is higher for patients with FHF than for patients with other indications for OLT.

204 Patients with FHF unlikely to survive without liver transplantation we

205 rom plasma of blood donors and patients with FHF, and from blood products (factor VIII and IX clottin

206 For patients with FHF, the RI group had a lower patient survival rate at 1

207 tion for antiviral therapy for patients with FHF.

208 s detected in four (19%) of 21 patients with FHF; in three cases, infection was detected at the onset