ライフサイエンスコーパス: FHL

1 FHL 124 cell FPR1 was atypical in that it resisted agoni

2 FHL Ia and group II afferents generally had increased di

3 FHL possesses functional nuclear localization and nuclea

4 FHL, which has the alpha drive of a classic extensor, re

5 FHL-1 is largely bound to Bruch's membrane through inter

6 FHL-1 promoted entry of Fba(+) group A streptococci into

7 roteins to factor H/factor H-like protein 1 (FHL-1) and other serum proteins from different animals w

8 Factor H and factor H like-protein 1 (FHL-1) are complement regulatory proteins that serve as

9 Treponema denticola factor H-like protein 1 (FHL-1) binding protein, FhbB, was recovered and characte

10 duces a factor H (FH) and FH-like protein 1 (FHL-1) binding protein.

11 s factor H (FH) and factor H-like protein 1 (FHL-1) for immune evasion.

12 s factor H (FH) and factor H-like protein 1 (FHL-1) for immune evasion.

13 uch as factor H and factor H-like protein 1 (FHL-1) for immune evasion.

14 protein A (FhbA) to bind FH-like protein 1 (FHL-1) has not been assessed previously.

15 CR7 of both CFH and factor H-like protein 1 (FHL-1), a splice variant of CFH (containing SCR1-7) with

16 ncated form of FH, called FH-like protein 1 (FHL-1), is the main regulatory protein in the layer of E

17 As expected, fH-like protein 1 (FHL-1), which contains fH SCR 6, also bound to fHbp-expr

18 ibitors factor H (FH) and FH-like protein 1 (FHL-1).

19 cally bound to factor H (FH)-like protein 1 (FHL-1).

20 s human factor H (FH) and FH-like protein 1 (FHL-1).

21 oteins factor H and factor H-like protein 1 (FHL-1).

22 ement, factor H and factor H-like protein 1 (FHL-1).

23 such as factor H (FH) and FH-like protein 1 (FHL-1).

24 ), and its splice product FH-like protein 1 (FHL-1; consisting of CCPs 1-7) are major regulators of t

25 proteins, Factor H, Factor H-like protein-1 (FHL-1), complement Factor H-related protein 1 (CFHR1), a

26 Retrospective review of 192 FHL tenograms and associated surgical records identified

27 ents with single heterozygous mutations in 2 FHL-associated genes.

28 l hemophagocytic lymphohistiocytosis (type 2 FHL, FHL2).

29 d as the disease-causing gene in FHL type 5 (FHL-5).

30 we report that SCPL-1 interacts with LIM-9 (FHL), a protein that we first discovered as an interacto

31 he M-line proteins UNC-89 (obscurin), LIM-9 (FHL), SCPL-1 (SCP), and UNC-96.

32 ge of C3b revealed that C3b is cleaved in an FHL-1/factor I-independent manner, perhaps by an unident

33 Fba is an FHL-1 and FH binding protein expressed on the surface of

34 Matrix contraction was determined using an FHL-124 patch contraction assay; at end-point, cells wer

35 the context of full-length CFH (SCR1-20) and FHL-1.

36 ons of the Y402 and H402 variants of CFH and FHL-1 with heparin, CRP, and several bacterial ligands:

37 In comparing the Y and H variants of CFH and FHL-1, we found no significant difference in their prote

38 e present study, we demonstrate that Fba and FHL-1 work in concert to promote invasion of epithelial

39 lates, indicating that Fba is a major FH and FHL-1 binding factor of serotype M1 streptococci.

40 Fba is an FH and FHL-1 binding protein expressed on the surface of the hu

41 f fba DNA sequences revealed that the FH and FHL-1 binding site in Fba is conserved among the M1 isol

42 n of fba greatly inhibited binding of FH and FHL-1 by all isolates, indicating that Fba is a major FH

43 at the role of Fba in recruitment of FH and FHL-1 by five serotype M1 isolates of streptococci.

44 purified FH as well as for binding of FH and FHL-1 from human plasma.

45 nstrated that B. hermsii absorbs both FH and FHL-1 from human serum.

46 , these data indicate that binding of FH and FHL-1 is a conserved function of Fba while modulation of

47 esting that the protease may modulate FH and FHL-1 recruitment during infection.

48 49 Fba protein was found to bind both FH and FHL-1.

49 eptococci have similar affinities for FH and FHL-1.

50 ce protein Fba can mediate binding of FH and FHL-1.

51 f FHDelta10-15 and midiFH to miniFH, FH, and FHL-1.

52 al for activating the expression of FHY1 and FHL (for FHY1-like), whose products are required for lig

53 at phyA may differentially regulate FHY1 and FHL activity through direct physical interaction and red

54 Although both FHY1 and FHL are capable of homo- and hetero-interaction via thei

55 ssisting phyA nuclear accumulation, FHY1 and FHL are required to assemble photoreceptor/transcription

56 Furthermore, we demonstrate that FHY1 and FHL directly interact with phyA by bimolecular fluoresce

57 est that the relative abundances of FHY1 and FHL in WT plants account for the differences in the seve

58 cence complementation and that both FHY1 and FHL interact more stably with the Pr form of phyA in Ara

59 tly activating the transcription of FHY1 and FHL, whose products are essential for light-induced phyA

60 transmit phyA signals downstream of FHY1 and FHL.

61 Factor H and FHL-1 displayed complement-regulatory activity, and boun

62 sed lipoprotein that binds both factor H and FHL-1.

63 lass II FHBPs (BBA68) bind both factor H and FHL-1.

64 Recombinant Gpd2 binds factor Hand and FHL-1, mainly via short consensus repeat 7; and binds pl

65 FHY1 (for FAR-RED ELONGATED HYPOCOTYL1) and FHL (for FHY1-LIKE).

66 1(+) Fba(+) streptococci preferentially bind FHL-1, whereas M1(-) Fba(+) streptococci have similar af

67 nstrate that T. denticola specifically binds FHL-1 via a 14-kDa, surface-exposed protein that we desi

68 rlaps the coiled-coil domain that binds both FHL-1 and FH.

69 s separated patients with perforin-deficient FHL from those with unidentified genetic cause(s) of the

70 Biallelic pathogenic mutations defining FHL were found in 171 (34%) patients; the proportion of

71 ulation have been more useful for diagnosing FHL than hemophagocytosis and the cytotoxicity assay.

72 Contrary to dogma, FHL-1 and FH exhibited equal regulatory activity, sugges

73 The four-and-a-half LIM domain (FHL) proteins are a family of LIM domain-only proteins i

74 Given this gene dosage effect, FHL is not strictly recessive.

75 common cause of FHL, we used an experimental FHL mouse model in which disease in perforin-deficient m

76 cells in the pathophysiology of experimental FHL.

77 Factor H and factor H-like protein 1 (FH/FHL-1) are soluble serum proteins that negatively regula

78 ors factor H and factor H-like protein 1 (FH/FHL-1).

79 lear how the interaction between CspA and FH/FHL-1 specifically enhances serum resistance.

80 lecular nature of the interaction between FH/FHL-1 and FhbA.

81 ria, including Borrelia burgdorferi, bind FH/FHL-1 on their cell surface to evade complement-mediated

82 face of B. burgdorferi that also can bind FH/FHL-1, it is presently unclear what role CRASP-1 plays i

83 While it is known that CspA binds FH/FHL-1, it is unclear how the interaction between CspA an

84 isolates (23/24) produced FhbA and bound FH/FHL-1.

85 One of the key B. burgdorferi FH/FHL-1 binding proteins identified thus far was designate

86 ed proteins were generated and tested for FH/FHL-1 binding.

87 hbA that may serve as a contact point for FH/FHL-1.

88 To determine if FH/FHL-1 binding is widespread among B. hermsii isolates, a

89 ce protein 1 (CRASP-1), encodes the major FH/FHL-1-binding protein of B. burgdorferi.

90 Binding of FH/FHL-1 by the B. burgdorferi proteins CspA and the OspE-r

91 The binding of FH/FHL-1 on the surface of B. burgdorferi is thought to enh

92 ollectively, these analyses indicate that FH/FHL-1 binding is a widespread virulence mechanism for B.

93 verse panel of strains was tested for the FH/FHL-1 binding phenotype and FhbA production.

94 in the formation and presentation of the FH/FHL-1-binding pocket.

95 lar basis of the interaction of FhbA with FH/FHL-1, recombinant FhbA truncated proteins were generate

96 As for FHY1, FHL transcript accumulation is dependent on FHY3 and is

97 pitation experiments showed that phyA, FHY1, FHL, LAF1, and HFR1 are components of protein complexes

98 be accounted for by a critical role for FHY1-FHL heterodimers in phyA signal transmission.

99 on domain mediates the formation of the FHY1/FHL/PHYA far-red-absorbing form complex, whereby it play

100 and peptide W revealed the same profile for FHL 124 cells, neutrophils, and FPR1-transfected HEK 293

101 A binding site for FHL-1 and FH was localized to the N-terminal half of Fba

102 e of ST2 as a novel therapeutic strategy for FHL.

103 th open reading frame in sequenced cDNA from FHL 124 cells.

104 omplement active serum depleted of Factor H, FHL-1, and CFHR1, demonstrating a protective role of the

105 Factor H, FHL-1, and plasminogen when bound to Lpd were functional

106 factor of P. aeruginosa that binds Factor H, FHL-1, CFHR1, and plasminogen, and the Lpd-attached regu

107 Familial forms of HLH (FHL), which are increasingly found also in adolescents a

108 is the membrane-bound formate hydrogenlyase (FHL) complex, which links formate oxidation to proton re

109 The formate hydrogenlyase (FHL) enzyme from Escherichia coli normally oxidizes form

110 These results identify FHL proteins as negative regulators of HIF-1 activity, w

111 ed reactive proliferation of CD8+ T cells in FHL, resulting in atypical morphology and unusual immuno

112 ntegrin (fibronectin receptor components) in FHL 124 cells and human lens epithelia.

113 nd MT1-MMP and induced matrix contraction in FHL-124 cells.

114 e mechanisms of hemopoietic dysregulation in FHL.

115 rucial amplifiers of immune dysregulation in FHL.

116 ced pertussis toxin-sensitive Ca(2+) flux in FHL 124 cells, consistent with classic G(i)-mediated FPR

117 en identified as the disease-causing gene in FHL type 5 (FHL-5).

118 TXBP2 in neutrophils, and for neutrophils in FHL in general, has not been documented thus far.

119 To localize the Fba binding site in FHL-1 and FH, surface plasmon resonance was used to asse

120 ent interleukin-33 (IL-33) receptor, ST2, in FHL.

121 20% increase in heart weight/body weight in FHL null versus wild-type mice; P<0.01).

122 nding site, and heparin was found to inhibit FHL-1 binding to Fba.

123 Consistent with its FHL-1 binding specificity, FhbB binds only to factor H r

124 dilution but no mutations in the other known FHL-related genes (PRF1, STXBP2, and UNC13D).

125 HYPOCOTYL1 (FHY1) and its homolog FHY1-LIKE (FHL) define two positive regulators in the phyA signalin

126 D ELONGATED HYPOCOTYL1 (FHY1) and FHY1-LIKE (FHL), and two transcription factors, LONG AFTER FAR-RED

127 A member of the four-and-a-half-LIM (FHL) domain protein family, FHL1, is highly expressed in

128 ntiation markers in the human lens cell line FHL 124 and native lens epithelia.

129 s, the human fetal lens epithelial cell line FHL 124 expressed FPR1 mRNA and was strongly FPR1 protei

130 The human lens epithelial cell-line FHL-124 and human capsular bag models were employed.

131 orin gene of eight unrelated 10q21-22-linked FHL patients revealed homozygous nonsense mutations in f

132 perforin are responsible for 10q21-22-linked FHL.

133 simotor drive to the flexor hallucis longus (FHL) and flexor digitorum longus (FDL) muscles during lo

134 dies, harvesting the flexor hallucis longus (FHL) tendon may cause nerve injury.

135 gists in humans, the flexor hallucis longus (FHL, a toe flexor) and the anal sphincter, as a model th

136 he pathogen (through formate hydrogen lyase [FHL] and Hyc) is insignificant in terms of providing res

137 familial hemophagocytic lymphohistiocytosis (FHL) forms.

138 Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically determined hyperinflammatory syndr

139 Familial hemophagocytic lymphohistiocytosis (FHL) is a life-threatening disorder of immune regulation

140 Familial hemophagocytic lymphohistiocytosis (FHL) is a rare and often fatal disorder characterized by

141 Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, genetically heterogeneous autosomal rece

142 Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, rapidly fatal, autosomal recessive immun

143 Familial hemophagocytic lymphohistiocytosis (FHL) is an inherited, fatal disorder of infancy.

144 Familial hemophagocytic lymphohistiocytosis (FHL) is caused by genetic defects in cytotoxic granule c

145 familial hemophagocytic lymphohistiocytosis (FHL) to screen for biologic correlates with the genetic

146 familial hemophagocytic lymphohistiocytosis (FHL), are lethal disorders caused by uncontrolled, syste

147 familial hemophagocytic lymphohistiocytosis (FHL), have various underlying genetic abnormalities, the

148 familial hemophagocytic lymphohistiocytosis (FHL).

149 familial hemophagocytic lymphohistiocytosis (FHL).

150 Here we identify a homolog of FHY1 named FHL (FHY1-like) as a novel signaling factor essential fo

151 T. denticola preferentially binds FH and not FHL-1, and that FH is then cleaved by dentilisin to yiel

152 eraction via their C-termini, the ability of FHL overexpression to restore wild-type (WT) morphologic

153 Analyses of the ability of FHL-1 bound to the surface of T. denticola to serve as a

154 nt to explain several fundamental aspects of FHL, namely, the inability of many pathogenic antigens t

155 ombinant proteins and assayed for binding of FHL-1 and FH by Western blotting, enzyme-linked immunoso

156 y, as evidenced by a low level of binding of FHL-1 relative to that of fH.

157 ncreased risk of AMD, reduces the binding of FHL-1 to this heparan sulfate.

158 netics, maturation, and some biochemistry of FHL are understood, the protein complex has never been i

159 UNC13D (FHL3) accounted for 70% of cases of FHL.

160 se mutation in perforin is a common cause of FHL, we used an experimental FHL mouse model in which di

161 nt step in understanding the contribution of FHL-1 binding in T. denticola pathogenesis and in develo

162 of the N2B spring element and expression of FHL proteins trigger cardiac hypertrophy.

163 ents with "late onset and relapsing" form of FHL related to B- and T-cell differentiation/survival, T

164 s with "late-onset" and "relapsing" forms of FHL from patients with an "early onset and rapidly evolv

165 13-4) underlie the other identified forms of FHL, we assessed whether syntaxin 11 might also serve a

166 we hypothesize that the primary function of FHL-1 binding by T. denticola might be to facilitate adh

167 atterns, which underlie the immunobiology of FHL.

168 s a potential digenic mode of inheritance of FHL as a result of a synergistic function effect within

169 reported that allow the facile isolation of FHL in a single chromatographic step.

170 tion of ST2 signaling in the murine model of FHL reduces T cell-mediated production of IFNgamma and s

171 In the murine model of FHL, in which perforin-deficient (Prf1(-/-)) mice are in

172 und in 171 (34%) patients; the proportion of FHL was much higher (64%) in patients given a diagnosis

173 In this work, the reverse reaction of FHL is unlocked.

174 latory activity, suggesting that the role of FHL-1 in AP regulation has been underestimated.

175 ed patients with unknown genetic cause(s) of FHL into 2 well-distinguished subgroups.

176 ish between genetic and clinical subtypes of FHL.

177 itivity to FR associated with suppression of FHL expression in fhy1-3 cannot be accounted for by a cr

178 ngation assays indicated that suppression of FHL expression in fhy1-3 caused an insensitivity of hypo

179 -terminal region of FHY1, as well as that of FHL, interacting with the LAF1 N-terminal portion and th

180 To advance the understanding of FHL, we performed gene expression profiling of periphera

181 gene response that includes upregulation of FHL proteins.

182 Lys revealed ~2500 specific binding sites on FHL-124 cells (K(D) ~ 0.5 nm) versus ~40,000 sites on ne

183 fection and binds factor H (fH) and possibly FHL-1/reconectin.

184 embers of the four and one-half LIM protein (FHL) family have been identified and cloned.

185 n of this coiled-coil domain greatly reduced FHL-1 and FH binding.

186 In agreement with previous reports, FHL gave a single burst of EMG activity during the step

187 yzed a cohort of 185 patients with suspected FHL for mutations in STXBP2.

188 are approximately 15-fold more abundant than FHL transcripts.

189 to generate anal sphincter contraction than FHL contraction.

190 It is established that FHL can operate as a highly efficient hydrogen-dependent

191 Linkage analyses indicate that FHL is genetically heterogeneous and linked to 9q21.3-22

192 Here, we report that FHL-5 neutrophils have a profound defect in granule mobi

193 We show that FHL-1 can passively diffuse through Bruch's membrane, wh

194 We also show that FHL-1 is retained in drusen whereas FH coats the periphe

195 describe functional interactions between the FHL proteins and HIF-1.

196 endon, due to the large distance between the FHL tendon and the medial and lateral plantar nerves.

197 with anesthetic injection helped confirm the FHL sheath as the pain generator.

198 e minimally invasive incision to harvest the FHL tendon, due to the large distance between the FHL te

199 A retraction was performed to harvest the FHL through the posteromedial hindfoot incision using a

200 As isolated, the FHL complex retains formate hydrogenlyase activity in vi

201 rstanding and harnessing the activity of the FHL complex is critical to advancing future biohydrogen

202 Expression of the FHL proteins increased upon HIF-1alpha induction, sugges

203 Tenography of the FHL sheath produced diagnostic images in almost all pati

204 Communication of the FHL sheath with the ankle, flexor digitorum longus, or s

205 , 43 had monoallelic mutations in one of the FHL-defining genes.

206 Thus, FHL-124 cells express functional FPR1, which is consiste

207 enticola might be to facilitate adherence to FHL-1 present on anchorage-dependent cells and in the ex

208 nsensus repeat 7 (SCR 7), a domain common to FHL-1 and FH.

209 onsistent with phasic dynamic gamma drive to FHL spindles linked with alpha drive.

210 In conclusion, the gamma drives to FHL and FDL differed during locomotion.

211 es from HLH secondary to severe infection to FHL.

212 ions in RAB27A, LYST, and AP3B1 give rise to FHL associated with oculocutaneous albinism, and patient

213 uclear cells from 11 children with untreated FHL.

214 ed H2 oxidation and formate production using FHL became an alternate route for electron disposal duri

215 eriorly that activates during both voluntary FHL contraction as well as voluntary anal sphincter cont

216 at preferentially activates during voluntary FHL contraction and one located more anteriorly that act

217 ic recordings, we demonstrate that voluntary FHL contraction is associated with synergistic anal sphi

218 Lines in which FHL function was abolished by insertional mutagenesis or

219 e-wide expression profiling in children with FHL demonstrates the complexity of gene expression patte

220 Comparisons between patients with FHL and normal pediatric controls (n = 30) identified 91

221 A number of patients with FHL and normal pigmentation remain without a genetic dia

222 h oculocutaneous albinism, and patients with FHL are usually only screened for mutations in these gen

223 We asked whether patients with FHL with immunodeficiency but with normal pigmentation m

224 need for RAB27A sequencing in patients with FHL with normal pigmentation and identify a critical bin

225 of RAB27A, LYST, and AP3B1 in patients with FHL with pigment dilution, as well as a cohort with no c

226 For patients with FHL, stem cell transplantation is indicated and can be c

227 testinal tract inflammation in patients with FHL-5.

228 proteins were overexpressed in patients with FHL.

229 tiation were down-regulated in patients with FHL.

230 ssible in more than 90% of our patients with FHL.

231 ase in growth rate relative to cells without FHL.

232 ry anal sphincter contraction occurs without FHL contraction.