ライフサイエンスコーパス: fibrillin

1 and -2 are deposited on and coregulated with fibrillin.

2 odomain binding targets the growth factor to fibrillin.

3 1, a ZP domain protein related to vertebrate fibrillins.

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

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

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

7 Partial fibrillin 1 gene inactivation in cardiomyocytes was suff

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

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

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

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

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

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

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

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

16 ity and the reexpression of endothelial cell fibrillin 1.

17 utations in the extracellular matrix protein fibrillin 1.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

65 Fibrillin-1 deficiency is associated with excess signali

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

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

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

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

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

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

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

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

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

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

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

77 Fibrillin-1 filaments splayed out, extending beyond the

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

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

80 Circulating fibrillin-1 fragments represent a new potential biomarke

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

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

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

84 Underlying fibrillin-1 gene mutations cause increased transforming

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

102 Fibrillin-1 microfibril assembly and secreted lysyl oxid

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

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

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

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

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

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

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

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

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

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

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

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

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

116 Fibrillin-1 mutations are believed to promote abnormal S

117 Fibrillin-1 mutations associated with Marfan syndrome ha

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

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

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

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

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

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

124 63 had high levels of FBN1 mRNA and secreted fibrillin-1 protein to form extracellular matrix fibres.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

183 ich encodes the extracellular matrix protein fibrillin-1.

184 cules including fibronectin, osteopontin and fibrillin-1.

185 equilibrium encompassing FBN1, which encodes fibrillin-1.

186 functional relationship between ADAMTS10 and fibrillin-1.

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

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

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

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

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

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

193 ne encoding the extracellular matrix protein fibrillin-1.

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

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

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

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

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

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

200 nd identified a rare variant p.Glu1144Lys in Fibrillin 2 (FBN2), a glycoprotein of the elastin-rich e

201 brils in cultured fibroblasts and suppresses fibrillin-2 (FBN2) incorporation in microfibrils, in par

202 d on these results, FBN1 and a related gene, fibrillin-2 (FBN2), were sequenced in a total of 852 AIS

203 re, we show that the corresponding region of fibrillin-2 binds heparin very poorly, highlighting a no

204 ADAMTS17 binds recombinant fibrillin-2 but not fibrillin-1 and does not cleave eith

205 We recently found that fibrillin-1 and fibrillin-2 control bone formation by regulating osteobl

206 Fibrillin-2 epitopes are also progressively revealed in

207 fined epitopes, demonstrated that N-terminal fibrillin-2 epitopes are masked in postnatal microfibril

208 vely revealed in these mice, suggesting that fibrillin-2 immunoreactivity can serve as a marker for m

209 ity of microfibrils to determine the role of fibrillin-2 in postnatal microfibril structure.

210 , these data demonstrate that involvement of fibrillin-2 in the initial assembly of the aortic matrix

211 ither TAA nor dissection was associated with fibrillin-2 or fibulin-4.

212 f immunolabeled ECM components (fibronectin, fibrillin-2) and TIE1 positive endocardial progenitors i

213 Concentrations of fibrillin-1, fibrillin-2, and fibulin-4 were measured with novel immu

214 Consistent with a specialized function of fibrillin-2, electron microscopy visualized ultrastructu

215 Mutations in fibrillin-1 or fibrillin-2, the major structural components of extracel

216 Fibrillin-2-null (Fbn2(-/-)) mice display a low bone mas

217 ssembly, including fibulin-4, fibulin-5, and fibrillin-2.

218 ich encodes the extracellular matrix protein Fibrillin 2b (OMIM ID: 121050).

219 s in candidate arthrogryposis-causing genes (fibrillin 3 [FBN3], myosin IXA [MYO9A], and pleckstrin a

220 lved in photosystem I assembly, and specific fibrillins, a flavin reductase-like protein, and an aldo

221 lasmon resonance, binding affinities between fibrillin and all propeptides were determined.

222 in our study reflect sequential unfolding of fibrillin and can explain the process of its reversible

223 nding EGF domains, related to the vertebrate Fibrillin and Fibulin gene families.

224 Important recent understandings of fibrillins and fibrillin-associated microfibril proteins

225 ple genetic disorders caused by mutations in fibrillins and in microfibril-associated molecules.

226 It has homology to fibrillins and may have roles in cell adhesion and as a

227 Microfibrillar proteins mainly include fibrillins and microfibril-associated glycoproteins (MAG

228 se data provide insights into the biology of fibrillins and the pathologies of WMS, AD and GD.

229 cellular microfibrillar networks composed of fibrillins and their associated ligands such as LTBPs, f

230 er TGFbeta-like growth factors interact with fibrillins and/or LTBPs and are also targeted to the ext

231 merging understandings of the effects of Tsk fibrillin, and genetic and autoimmune studies of human f

232 d in fibrosis, including multiple collagens, fibrillins, and elastin.

233 The fibrillins are a large family of chloroplast proteins th

234 Fibrillins are also expressed in embryos, but no early d

235 owth factor-beta (TGF-beta)-binding proteins fibrillins are components of microfibril networks, and b

236 o and ex vivo experiments that implicate the fibrillins as negative regulators of bone resorption.

237 tant recent understandings of fibrillins and fibrillin-associated microfibril proteins suggest new wa

238 Fibrillin-based human diseases such as Marfan syndrome a

239 -rich low molecular weight components of the fibrillin-based microfibrillar complex.

240 in the fibrillin-1 structure from mutant Tsk fibrillin cause hypodermal fibrosis and associated chang

241 the dermis in which hyaluronan (HA)-versican-fibrillin complexes are found.

242 ic properties and structural organization of fibrillin-containing microfibrils are based primarily on

243 Disruption of fibrillin-1 assembly by MFS fibrillin decreased CCN3 expression and skin from patien

244 Fibrillin expression was analyzed by in situ hybridizati

245 ng protein 1 (LTBP1) is a member of the LTBP/fibrillin family of extracellular proteins.

246 Plastoglobulins (PGL) also known as fibrillins (FBN) are evolutionary conserved proteins pre

247 re of gastrulation associated with defective fibrillin fibril assembly.

248 nt with a dominant-negative effect on native fibrillin fibril assembly.

249 s positive signals from TGF-beta and Wnt for fibrillin fibrillogenesis and profibrotic gene expressio

250 f aneurysm patients had detectable levels of fibrillin fragments.

251 These changes may reflect the unraveling of fibrillin from the complex folded arrangement into a lin

252 hat each contained at least one ABC1K and/or fibrillin gene.

253 enital contractural arachnodactyly implicate fibrillins in the function and homeostasis of multiple a

254 gen-1A1 (COL1A1), collagen-3A1 (COL3A1), and fibrillin increased significantly in CHF fibroblasts.

255 imultaneous N-terminal matrix and C-terminal fibrillin interactions providing tethering for TGFbeta a

256 ogether, these data show that native Xenopus fibrillin is essential for the process of directed conve

257 Fibrillin is placed centrally not only as the primary st

258 and other organs, where it may regulate the fibrillin isoform composition of microfibrils.

259 Fibrillin may thus communicate alterations in matrix to

260 ity that latent TGFbeta-binding proteins and fibrillins may mediate interactions with all other prodo

261 TS-like protein which has been implicated in fibrillin microfibril biogenesis, cause ectopia lentis (

262 Marchesani syndrome (WMS), implicating it in fibrillin microfibril biology since some fibrillin-1 mut

263 These data implicate the fibrillin microfibril network in the extracellular contr

264 Improved understanding of how fibrillin microfibrils and associated proteins regulated

265 Fibrillin microfibrils are 10-12 nm diameter, extracellu

266 Fibrillin microfibrils are polymeric structures present

267 oire of fibrillin strengthens the concept of fibrillin microfibrils as extracellular scaffolds integr

268 Fibrillin microfibrils form the ocular zonule and are pr

269 Fibrillin microfibrils have essential roles in elastic f

270 showed that EMILIN-1 and -2 are targeted to fibrillin microfibrils in the skin.

271 A role for fibrillin microfibrils in tissue elasticity has been imp

272 The importance of fibrillin microfibrils to connective tissue function has

273 own about this protease or its connection to fibrillin microfibrils, whose major component, fibrillin

274 This finding supports a pleating model where fibrillin molecules are highly folded within the microfi

275 astic properties lies in the organization of fibrillin molecules, which, unfortunately, is still poor

276 Assembly of fibrillin monomers into microfibrils is thought to occur

277 This review discusses mutant-fibrillin mouse models of Marfan syndrome and SSc (Tsk m

278 sion of miR29b decreased COL1A1, COL3A1, and fibrillin mRNA by 65%, 62%, and 61% (all p<0.001), respe

279 (-68%; p<0.01) enhanced COL1A1, COL3A1, and fibrillin mRNA expression by 28% (p<0.01), 19% (p<0.05),

280 mediates at least part of the effect of Tsk fibrillin on CCN3 which is consistent with a synergistic

281 and genetic and autoimmune studies of human fibrillin on dermal fibrosis.

282 From these studies, we conclude that fetal fibrillin polymers form an inner core within postnatal m

283 c patient populations, and autoantibodies to fibrillin provide potential links to human SSc.

284 roteome of chloroplast PGs consists of seven fibrillins, providing a protein coat and preventing coal

285 ains of BMP-2, -4, -7, and -10 and GDF-5 and fibrillins, raising the possibility that latent TGFbeta-

286 rall, these results expand our definition of fibrillin-related disorders to include AIS and open up n

287 rks, suggesting an involvement of EMILINs in fibrillin-related skin disorders.

288 eover, TH treatment increased intracutaneous fibrillin-rich microfibril and collagen III deposition a

289 is a small molecular weight component of the fibrillin-rich microfibril.

290 Fibrillin-rich microfibrils are extracellular assemblies

291 Fibrillin-rich microfibrils are the major structural com

292 An X-ray diffraction study of hydrated fibrillin-rich microfibrils from zonular filaments has b

293 To elucidate the contribution of fibrillin-rich microfibrils to organogenesis, we have ex

294 in vertebrates, is a ubiquitous component of fibrillin-rich microfibrils.

295 ed protein, plastid-lipid-associated protein-fibrillins, SOUL heme-binding proteins, phytyl ester syn

296 ition of EMILINs to the ligand repertoire of fibrillin strengthens the concept of fibrillin microfibr

297 ins, a family of molecules homologous to the fibrillins, the prodomain of BMP-7 was tested for bindin

298 oteins, including six ABC1 kinases and seven fibrillins together comprising more than 70% of the PG p

299 Other fibrillins were located predominantly in the stroma or t

300 pendent methods to reveal a role for Xenopus fibrillin (XF) at gastrulation.