ライフサイエンスコーパス: fibrillin-1

1 regulated, including decorin, fibulin 1, and fibrillin 1.

2 ity and the reexpression of endothelial cell fibrillin 1.

3 utations in the extracellular matrix protein fibrillin 1.

4 the connective-tissue microfibrillar protein fibrillin 1.

5 ce (IF) using a monoclonal antibody (mAb) to fibrillin 1.

6 ich encodes the extracellular matrix protein fibrillin-1.

7 nisms due to haploinsufficiency of wild-type fibrillin-1.

8 beta-binding proteins (LTBPs) interact with fibrillin-1.

9 fibulins 4 and 5, lysyl oxidase like-1, and fibrillin-1.

10 dicate differences in their binding sites in fibrillin-1.

11 binding site within the N-terminal domain in fibrillin-1.

12 ne encoding the extracellular matrix protein fibrillin-1.

13 e caused by deficiency of the matrix protein fibrillin-1.

14 large latent complex [LLC]) with N-terminal fibrillin-1.

15 caused by mutations in the gene that encodes fibrillin-1.

16 eine-rich extracellular matrix (ECM) protein fibrillin-1.

17 r dimer, interact with N-terminal regions of fibrillin-1.

18 levels may result in an altered function of fibrillin-1.

19 ctive tissue disorder caused by mutations in fibrillin-1.

20 2, respectively, were shown to interact with fibrillin-1.

21 the structure of a homologous TB domain from fibrillin-1.

22 ll attachments that are normally mediated by fibrillin-1.

23 In family ANB, FAA was linked to fibrillin-1.

24 ctions prepared for immunohistochemistry for fibrillin-1.

25 rmis and to nondenatured versican but not to fibrillin-1.

26 cules including fibronectin, osteopontin and fibrillin-1.

27 equilibrium encompassing FBN1, which encodes fibrillin-1.

28 functional relationship between ADAMTS10 and fibrillin-1.

29 rminus and another in the C-terminal half of fibrillin-1.

30 anism that is controlled by the ECM molecule fibrillin-1.

31 Mutations in fibrillin 1, a key component of extracellular microfibri

32 Fibrillin 1, a large glycoprotein enriched in force-bear

33 A recent study implicates fibrillin-1, a component of extracellular matrix microfi

34 Sc-specific autoantibody against portions of fibrillin-1, a major component of ECM microfibrils and r

35 Fibrillin-1, a major component of microfibrils in the EC

36 sed by mutations in the gene (FBN1) encoding fibrillin-1, a major constituent of extracellular microf

37 in-1, and on known antibody binding sites in fibrillin-1, a model is proposed in which fibrillin-1 mo

38 ibrillin-1, or (2) dominant negative, normal fibrillin-1 abundance with mutant fibrillin-1 incorporat

39 TGF-beta inhibition rescued abnormalities in fibrillin-1 accumulation and matrix metalloproteinase ex

40 seen in Marfan aortas, including defects in fibrillin-1 accumulation, extracellular matrix degradati

41 , joint stiffness and ocular defects, whilst fibrillin-1 AD and GD have severe short stature, joint d

42 yzed the lung phenotype of mice deficient in fibrillin-1, an accepted model of Marfan syndrome.

43 in mice that were haploinsufficient for both fibrillin 1 and beta1 integrin.

44 rate that MAGP-2 specifically interacts with fibrillin-1 and -2 and suggest that MAGP-2 may help regu

45 Deletion analysis of partial fibrillin-1 and -2 clones revealed a calcium-binding epi

46 P-5 interacts with the N-terminal regions of fibrillin-1 and -2 in a site similar to the binding site

47 l. advance this concept by demonstrating how fibrillin-1 and -2 regulate TGF-beta and bone morphogene

48 at MFAP4 specifically binds tropoelastin and fibrillin-1 and -2, as well as the elastin cross-linking

49 In this study, we demonstrate that fibrillin-1 and -2, the structural components of extrace

50 ded known microfibrillar proteins, including fibrillin-1 and -2.

51 a-binding protein-1 and TGF-beta and between fibrillin-1 and bone morphogenetic protein-7 (BMP-7) are

52 AMTS17 binds recombinant fibrillin-2 but not fibrillin-1 and does not cleave either.

53 ssociated with aneurysm formation, including fibrillin-1 and FAA1.

54 We recently found that fibrillin-1 and fibrillin-2 control bone formation by re

55 Fibrillin-1 and fibrillin-2, encoded by FBN1 on chromoso

56 nto the medium and can differentiate between fibrillin-1 and fibrillin-2.

57 nective tissue was associated with increased fibrillin-1 and fibulin-2 matrices.

58 Nevertheless, both the detailed structure of fibrillin-1 and its organization within microfibrils are

59 t this digestion resulted in the cleavage of fibrillin-1 and loss of specific immunoreactive epitopes

60 n, oxytalan, and elastin-associated proteins fibrillin-1 and microfibrillar-associated protein-1/2 we

61 y of the aortic matrix overlaps in part with fibrillin-1 and that continued fibrillin-1 deposition is

62 created strain of mice that completely lacks fibrillin-1 and the consequences of combined deficiency

63 Wnt stimulate fibrillin-1 assembly and that fibrillin-1 and the developmental regulator CCN3 are bot

64 fibrillin-1 compete for interactions between fibrillin-1 and these fibulins.

65 g to analyze the solution structure of human fibrillin-1 and to produce ab initio structures of overl

66 los type IV syndromes, due to defects in the fibrillin-1 and type III procollagen genes, respectively

67 d the consequences of combined deficiency of fibrillins 1 and 2 on tissue formation.

68 The results demonstrated that fibrillins 1 and 2 perform partially overlapping functio

69 of this region prevents SS4 from binding to fibrillins 1 and alters SS4 localization in the chloropl

70 We showed previously that SS4 interacts with fibrillins 1 and is associated with plastoglobules, subo

71 phenotypes associated with Marfan syndrome (fibrillin-1) and congenital contractural arachnodactyly

72 th factor-like (cbEGF) domain fragments from fibrillin-1, and an unrelated protein Notch1, to analyse

73 xtensively in distribution with fibronectin, fibrillin-1, and MAGP-1 and moderately with decorin and

74 performed using antibodies against elastin, fibrillin-1, and microfibrillar-associated protein-1/2.

75 ude collagenase cleavage sites identified in fibrillin-1, and on known antibody binding sites in fibr

76 CMs) and promotes the assembly of collagens, fibrillin-1, and other proteins.

77 Mutations in fibrillin-1 are associated with thoracic aortic aneurysm

78 ts demonstrated that while normal amounts of Fibrillin-1 are synthesized, the protein itself appears

79 oding the extracellular matrix (ECM) protein fibrillin 1, are unusually vulnerable to stress-induced

80 1129Y, expressed as recombinant fragments of fibrillin-1, are retained and accumulate within the cell

81 To ascertain the clinical relevance of using fibrillin-1 as a marker for photoaging, facial skin was

82 he utility of potential repair agents, using fibrillin-1 as the marker for outcome.

83 the synthesis, secretion, and processing of fibrillin 1, as well as to observe microfibril formation

84 CCN3 overexpression markedly repressed fibrillin-1 assembly and also blocked other TGFbeta- and

85 iously shown that TGF-beta and Wnt stimulate fibrillin-1 assembly and that fibrillin-1 and the develo

86 Disruption of fibrillin-1 assembly by MFS fibrillin decreased CCN3 exp

87 These data indicate that anti-fibrillin-1 autoantibodies can induce the activation of

88 examine a potential pathogenic role of anti-fibrillin-1 autoantibodies, normal human fibroblasts wer

89 nished the activation of fibroblasts by anti-fibrillin-1 autoantibodies.

90 in medium containing normal human IgG, anti-fibrillin-1 autoantibody-treated normal dermal fibroblas

91 retain normal domain structure and keep the fibrillin-1-binding site intact, none of these mutant pr

92 Both pulse chase analysis of fibrillin-1 biosynthesis and RNase protection analyses d

93 igand in cbEGF32, does not detectably affect fibrillin-1 biosynthesis, rate of secretion, processing,

94 Furin-activated ADAMTS10 could cleave fibrillin-1, but innate resistance of ADAMTS10 zymogen t

95 These data show for the first time that fibrillin-1, but not fibulin-2 or fibulin-4, is required

96 does not disrupt the secretion of endogenous fibrillin-1 by the cell, we propose that G1127S causes d

97 ss a disease-associated mutant form of human fibrillin-1 (C1663R) on a normal mouse background.

98 Furthermore, fibrillin-1 can be used as a "reporter" molecule in shor

99 ve to be the first demonstration that mutant fibrillin-1 can participate in productive microfibrillar

100 To test the hypothesis that mutations in fibrillin-1 cause disorders through primary effects on m

101 Most mutations in fibrillin-1 cause Marfan syndrome with severe cardiovasc

102 While most of the known mutations in fibrillin-1 cause Marfan syndrome, a number of other mut

103 These results suggest that Tsk fibrillin-1 causes skin tethering by altering matrix pro

104 py was performed to investigate ADAMTSL4 and fibrillin-1 colocalization in these cultures.

105 differences, interactions between LTBP-1 and fibrillin-1 compete for interactions between fibrillin-1

106 the highest compared with lowest quartile of fibrillin-1 concentration (OR=2.9; 95% CI, 1.6-5.0).

107 the highest compared with lowest quartile of fibrillin-1 concentration.

108 scopy of molecules of BMP-7 complex bound to fibrillin-1 confirmed these findings and also showed tha

109 It colocalizes to fibrillin-1 containing microfibrils in cultured fibrobla

110 skin of 12 SSc patients were used to examine fibrillin 1-containing microfibrils by immunofluorescenc

111 table, suggesting that an inherent defect of fibrillin 1-containing microfibrils may play a role in t

112 ibroblasts displayed a prominent meshwork of fibrillin 1-containing microfibrils when visualized by I

113 unoelectron microscopy localized ADAMTS10 to fibrillin-1-containing microfibrils in human tissues.

114 ng the release of sequestered TGF-beta1 from fibrillin-1-containing microfibrils in the ECM.

115 trans retinoic acid can significantly affect fibrillin-1 content in photoaged skin.

116 ues within the first hybrid domain (Hyb1) of fibrillin-1 contribute to interactions with LTBP-1 and L

117 Fibrillin-1 contributes to the regulated activation of t

118 Recently, we found that fibrillin-1 deficiency in mice impairs alveolar formatio

119 Fibrillin-1 deficiency is associated with excess signali

120 It was recently demonstrated that fibrillin-1 deficiency is associated with upregulation o

121 e pathogenetic sequence that is initiated by fibrillin-1 deficiency.

122 , and enhanced signaling is a consequence of fibrillin-1 deficiency.

123 ermined that dilated cardiomyopathy (DCM) in fibrillin 1-deficient mice is a primary manifestation re

124 expression profiling analysis comparing the fibrillin-1-deficient and wild-type developing lung.

125 Mitral valves from fibrillin-1-deficient mice exhibited postnatally acquire

126 Disruption of microfibrils in fibrillin-1-deficient mice leads to fragmentation of the

127 ivity leads to failed muscle regeneration in fibrillin-1-deficient mice.

128 ith other epidermal growth factor repeats in fibrillin-1, demonstrating specificity of the interactio

129 ine nuchal ligament cells showed accelerated fibrillin-1 deposition in ECM.

130 Immunofluorescence was used to evaluate fibrillin-1 deposition in the ECM of fetal bovine nuchal

131 Enhanced fibrillin-1 deposition in the presence of ADAMTSL4 and c

132 in part with fibrillin-1 and that continued fibrillin-1 deposition is absolutely required for the ma

133 We found that loss of fibrillin-1 deposition promotes the production of intrac

134 Aged mice deficient in fibrillin-1 develop destructive emphysema consistent wit

135 We show that substitutions in fibrillin-1 domains TB4 and TB5 that cause SSS and the a

136 of connective tissue caused by mutations in fibrillin-1 (encoded by FBN1 in humans and Fbn1 in mice)

137 anidine-extracted microfibrils contained all fibrillin-1 epitopes recognized by available antibodies.

138 FS), caused by a deficiency of extracellular fibrillin-1, exhibit myopathy and often are unable to in

139 trong correlation between increased CCN3 and fibrillin-1 expression, suggesting that CCN3 regulation

140 pression, suggesting that CCN3 regulation by fibrillin-1 extends to these CTDs.

141 the structure, expression, and deposition of fibrillin 1 (Fbn-1), type I collagen, and MAGP-2.

142 osis is caused by an in-frame duplication in fibrillin-1 (Fbn-1).

143 In humans, mutations in the gene for fibrillin 1 (FBN1) underlie Marfan syndrome (MS), a diso

144 sis using exome sequence data, we identified fibrillin-1 (FBN1) as the most significantly associated

145 A nonsense mutation in the fibrillin-1 (FBN1) gene of a Marfan syndrome (MFS) patie

146 However, in fibrillin-1 (Fbn1) null fibroblast cultures, LTBP-1 and

147 Mutations in fibrillin-1 (FBN1) result in Marfan syndrome, demonstrat

148 r caused by mutations in the gene coding for FIBRILLIN-1 (FBN1), an extracellular matrix protein.

149 MFS is caused by mutations in fibrillin-1 (FBN1), which encodes an extracellular matri

150 der caused by mutations in the gene encoding fibrillin-1 (FBN1).

151 vestigate the inner structure of the elastin-fibrillin-1 fibre network.

152 Concentrations of fibrillin-1, fibrillin-2, and fibulin-4 were measured wi

153 composed of several glycoproteins, including fibrillin-1, fibrillin-2, and MAGP1/2 (microfibril-assoc

154 ssociated with aneurysm formation, including fibrillin-1, fibrillin-2, and type III procollagen, and

155 In addition to fibrillin-1, fibronectin, vitronectin, laminin, and amyl

156 of major elastic fiber components (elastin, fibrillin-1, fibulin-4), collagens (types I, III, and IV

157 Fibrillin-1 filaments splayed out, extending beyond the

158 observed at the protein and mRNA levels for fibrillin-1 following all-trans retinoic acid and sodium

159 The extracellular glycoprotein fibrillin-1 forms microfibrils that act as the template

160 Mass spectrometry revealed that a fibrillin-1 fragment containing the TGFbeta1-releasing s

161 Circulating fibrillin-1 fragments represent a new potential biomarke

162 and the acromelic dysplasias do not prevent fibrillin-1 from being secreted or assembled into microf

163 requirement for the secretion of full-length fibrillin-1 from cells; (ii) failure to cleave off the C

164 e effect of misfolding on the trafficking of fibrillin-1 from fibroblast cells.

165 M haplotype on chromosome 15q containing the fibrillin 1 gene (FBN1) was strongly associated with sys

166 connective tissue caused by mutations in the fibrillin 1 gene (FBN1).

167 Partial fibrillin 1 gene inactivation in cardiomyocytes was suff

168 Mutations in the fibrillin-1 gene (FBN1) cause Marfan syndrome and relate

169 of SSc bears an in-frame duplication of the Fibrillin-1 gene (FBN1) which results in a larger than n

170 syndrome (MFS) is caused by mutations in the fibrillin-1 gene and dysregulation of transforming growt

171 Underlying fibrillin-1 gene mutations cause increased transforming

172 he majority of mutations affecting the human fibrillin-1 gene, FBN1, result in Marfan syndrome (MFS),

173 connective tissue caused by mutations in the fibrillin-1 gene, in which the patient presented with se

174 an 135 mutations have been identified in the fibrillin-1 gene, localized on chromosome 15q21.1 [corre

175 ening associated with expression of a mutant fibrillin-1 gene.

176 create a recombinant, GFP-tagged version of fibrillin-1 (GFP-Fbn) to study this process.

177 Moreover, autoantibodies specific for Fibrillin-1 have been demonstrated in serum from SSc pat

178 We show that mice deficient in fibrillin-1 have marked dysregulation of transforming gr

179 ly, metabolic studies indicated a paucity of fibrillin 1 in the ECM in the majority of the SSc fibrob

180 minished at a faster rate than the amount of fibrillin 1 in the ECM of control cells with time.

181 n vitro studies indicated that the amount of fibrillin 1 in the ECM of SSc cells diminished at a fast

182 Collectively, these findings implicate fibrillin 1 in the physiological adaptation of cardiac m

183 he lack of the high molecular weight form of fibrillin 1 in the SSc fibroblasts of Choctaw American I

184 nce specifically associates with full-length fibrillin-1 in cell layers.

185 Metabolic labeling studies of Fibrillin-1 in human SSc dermal fibroblasts demonstrated

186 nome-wide association studies also implicate fibrillin-1 in sporadic TAA.

187 ADAMTSL4 and colocalization of ADAMTSL4 with fibrillin-1 in the ECM of cultured fibroblasts suggest a

188 Genetic mutations in fibrillin-1, in a higher frequency in SSc patient popula

189 tituents of elastic fibers, tropoelastin and fibrillin-1, in vitro and localizes to elastic fibers in

190 ve, normal fibrillin-1 abundance with mutant fibrillin-1 incorporated in the matrix.

191 nt protein, allowing visualization of mutant fibrillin-1 incorporated into microfibrils.

192 We further show that Magp1 and fibrillin-1 interact in vivo.

193 ges and direct evidence that mouse and human fibrillin-1 interact with high efficiency.

194 C-terminal propeptide blocks the assembly of fibrillin-1 into microfibrils produced by dermal fibrobl

195 tion, processing, or deposition of reducible fibrillin-1 into the ECM.

196 and incorporation of full-length, GFP-tagged fibrillin-1 into the extracellular matrix, we investigat

197 ating that keratinocytes selectively secrete fibrillin-1 into the matrix and not into the medium and

198 Fibrillin-1 is a 330-kDa multidomain extracellular matri

199 Fibrillin-1 is a large modular glycoprotein that assembl

200 Fibrillin-1 is a member of the calcium-binding EGF repea

201 Human fibrillin-1 is an extra-cellular matrix glycoprotein wit

202 further suggests that the N-terminal half of fibrillin-1 is asymmetrically exposed in the outer filam

203 Most previous studies have focused on how fibrillin-1 is organized within microfibril polymers.

204 er filaments, whereas the C-terminal half of fibrillin-1 is present in the interior of the microfibri

205 Fibrillin-1 is the major component of the 10-12 nm diame

206 brillin microfibrils, whose major component, fibrillin-1, is genetically associated with ectopia lent

207 n and a reduced interaction with elastin and fibrillin-1 leading to impaired elastic fiber developmen

208 We show that Tsk fibrillin-1 leads to marked alterations in elastic fiber

209 n2) null or fibulin-4 (Fbln4) null cultures, fibrillin-1, LTBP-1, and LTBP-4 are incorporated into mi

210 g microfibrils when visualized by IF using a fibrillin 1 mAb.

211 Fibronectin, fibrillin-1, MAGP-1, decorin, and type VI collagen were

212 like fibers, codistributed with fibronectin, fibrillin-1, MAGP-1, decorin, and type VI collagen.

213 n did not change levels of matrix-associated fibrillin-1, MAGP-1, fibulin-2, fibulin-5, or emilin-1,

214 Previous studies have suggested that fibrillin 1 mediates skin fibrosis via its interface wit

215 Fibrillin-1 microfibril assembly and secreted lysyl oxid

216 xogenously added ADAMTS10 led to accelerated fibrillin-1 microfibril biogenesis.

217 in, laminin, vitronectin, tenascin, elastin, fibrillin-1, microfibril-associated glycoprotein (MAGP)-

218 in, laminin, vitronectin, tenascin, elastin, fibrillin-1, microfibril-associated glycoprotein (MAGP)-

219 sis of mouse mutations has demonstrated that fibrillin-1 microfibrils are mainly engaged in tissue ho

220 ADAMTSL4 colocalized with fibrillin-1 microfibrils in the ECM of these cells.

221 me patient with ADAMTS10 mutations deposited fibrillin-1 microfibrils sparsely compared with unaffect

222 hether ADAMTSL4 influences the biogenesis of fibrillin-1 microfibrils, which compose the zonule.

223 ulin-5 also showed impaired association with fibrillin-1 microfibrils.

224 beta complexes containing LTBP-3 with mutant fibrillin-1 microfibrils.

225 plication in the microfibrillar glycoprotein fibrillin-1, might show whether matrix alterations are s

226 One mutation leads to a truncated fibrillin-1 molecule that is tagged with green fluoresce

227 ghted a very compact, globular region of the fibrillin-1 molecule, which contains the integrin and he

228 s in stable microfibrils, demonstrating that fibrillin-1 molecules are not required to be in perfect

229 in fibrillin-1, a model is proposed in which fibrillin-1 molecules are staggered in microfibrils.

230 ound to molecules comigrating with authentic fibrillin-1 monomers in an assay using cell culture medi

231 CD44, tenascin C and fibrillin-1 mRNA levels were reduced by 4MU treatment, b

232 data demonstrate that during biosynthesis of fibrillin-1, multiple tandem repeats of cbEGF domains ma

233 y and could ameliorate disease phenotypes in fibrillin-1 mutated systemic sclerosis (SS) and dextran-

234 in fibrillin microfibril biology since some fibrillin-1 mutations also cause WMS.

235 Fibrillin-1 mutations are believed to promote abnormal S

236 Fibrillin-1 mutations associated with Marfan syndrome ha

237 The demonstration that fibrillin-1 mutations perturb transforming growth factor

238 sults obtained from studies of wild type and fibrillin-1 null tissues, using monoclonal and polyclona

239 hat these proteins mediate the effect of Tsk-fibrillin-1 on elastogenesis.

240 Mutations in fibrillin-1 or fibrillin-2, the major structural compone

241 prodomain of BMP-7 was tested for binding to fibrillin-1 or to LTBP-1.

242 attachment and self-renewal of hESCs alone (fibrillin-1) or in combination with fibronectin (perleca

243 ploinsufficiency, decreased amount of normal fibrillin-1, or (2) dominant negative, normal fibrillin-

244 ted to test whether circulating fragments of fibrillin-1, or other microfibril fragments, are associa

245 he effects of homocysteine are not unique to fibrillin-1, other cbEGF-containing proteins may be impl

246 Modulation of binding affinities by fibrillin-1 polypeptides in which residues in the third

247 cid desmosine, and that it co-localizes with fibrillin-1-positive fibers in vivo.

248 e examined the abundance and distribution of fibrillin-1 prior to, and following, 192 wk of all-trans

249 stead, triiodothyronine increased sirtuin-1, fibrillin-1, proliferator-activated receptor-gamma 1-alp

250 DECs demonstrated the aberrant expression of fibrillin 1 protein only in apoptotic endothelial cells

251 46,XXdel(15)(q15q22.1)] was identified whose fibrillin-1 protein and mRNA levels were significantly h

252 port in this issue now proposes that loss of fibrillin-1 protein by any of several mechanisms and the

253 FBN1 mutations were classified based on fibrillin-1 protein effect into (1) haploinsufficiency,

254 63 had high levels of FBN1 mRNA and secreted fibrillin-1 protein to form extracellular matrix fibres.

255 old lower and produced negligible amounts of fibrillin-1 protein.

256 utations that affect specific domains of the fibrillin-1 protein.

257 model that invokes haploinsufficiency for WT fibrillin-1, rather than production of mutant protein, a

258 ouring analogous amino acid substitutions in fibrillin-1 recapitulate aggressive skin fibrosis that i

259 This region is distinct from the region of fibrillin-1 reported by others to bind MAGP-1.

260 In humans, mutations in fibrillin-1 result in a variety of genetic disorders wit

261 e, encoding the extracellular matrix protein fibrillin-1, result in the dominant connective tissue di

262 rame deletion of the first hybrid domain) in fibrillin-1 results in stable microfibrils, demonstratin

263 the dramatic paucity of matrix-incorporated fibrillin-1 seen in heterozygous patient samples.

264 By releasing LLC from microfibrils, the fibrillin-1 sequence encoded by exons 44-49 can contribu

265 esence of cell layer extracellular matrix, a fibrillin-1 sequence encoded by exons 44-49 releases end

266 studies revealed that the N-terminal end of fibrillin-1 serves as a universal high affinity docking

267 ot the adult DM was positive for tenascin-C, fibrillin-1, SPARC, and laminin-332.

268 cent studies suggest that alterations in the fibrillin-1 structure from mutant Tsk fibrillin cause hy

269 ed the absence of LTBP-3 in matrices lacking fibrillin-1, suggesting that perturbed TGFbeta signaling

270 Comparison with the fibrillin-1 TB domain reveals that the two-residue inser

271 ins a two amino acid insertion, not found in fibrillin-1 TB domains, which is not amenable to molecul

272 arin/heparan sulfate binding to two sites on fibrillin-1 TB5 using a mutagenesis approach.

273 mutations all localize to the only domain in fibrillin-1 that harbours an Arg-Gly-Asp (RGD) motif nee

274 sed by structural or quantitative defects in fibrillin-1 that perturb tissue integrity and TGFbeta bi

275 opposite those associated with mutations in fibrillin-1 that result in enhanced TGF-beta signaling.

276 d by mutations of the microfibrillar protein fibrillin-1, that predisposes affected individuals to ao

277 ree domains of the LTBP-1 C terminus, and in fibrillin-1 the site was defined within four domains nea

278 However, upon binding to fibrillin-1, the BMP-7 complex is rendered into a closed

279 ese data show that upon prodomain binding to fibrillin-1, the BMP-7 complex undergoes a conformationa

280 are the two major structural motifs found in fibrillin-1, the extracellular matrix (ECM) protein defe

281 ssense mutations in the gene (FBN1) encoding fibrillin-1, the main constituent of extracellular micro

282 om mutations in the FBN1 gene, which encodes fibrillin-1, the precise mechanism by which the pleiotro

283 ts strongly and specifically with N-terminal fibrillin-1, thereby inhibiting the association of C-ter

284 unolocalization demonstrated the presence of fibrillin-1 throughout the lamina densa in the dermal--

285 tes in microfibril biogenesis rather than in fibrillin-1 turnover.

286 n of a novel cryptic site present in EGF4 in fibrillin-1 underscores the molecular complexity and tis

287 cretion and microfibril assembly profiles of fibrillin-1 variants containing substitutions associated

288 Specifically, fibrillin-1 was investigated as a potential ADAMTS10 bin

289 Fibrillin-1 was located at the base and lateral edges of

290 In particular, a high frequency of anti-Fibrillin-1 was observed in Japanese patients with diffu

291 (MAGP-2), a protein that is associated with fibrillin 1, was altered in the skin of patients with SS

292 with that of the non-TGF-beta-binding 8-Cys6(fibrillin-1), we observed that a two-residue insertion i

293 ase caspase 3 as well as the SSc autoantigen fibrillin 1 were demonstrated.

294 cilin with molecules such as fibronectin and fibrillin-1 were confirmed biochemically.

295 regions near the second 8-cysteine domain in fibrillin-1 were easily cleaved by crude collagenase.

296 Two sites of ADAMTS10 binding to fibrillin-1 were identified, one toward the N terminus a

297 th SS4 localization and its interaction with fibrillins 1 were mediated by the N-terminal part of SS4

298 We have discovered that fibrillin-1, which forms extracellular microfibrils, can

299 secreted metalloprotease) and FBN1 (encoding fibrillin-1, which forms tissue microfibrils), respectiv

300 e, recombinant ADAMTS10 was found to bind to fibrillin-1 with a high degree of specificity and with h