ライフサイエンスコーパス: fibrillin

1 odomain binding targets the growth factor to fibrillin.

2 and -2 are deposited on and coregulated with 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 binding protein 1 and influence assembly of fibrillin 1 microfibrils.

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

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

12 BN1 encodes the extracellular matrix protein fibrillin 1, which is a major structural component of mi

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

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

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

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

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

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

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

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

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

22 tissue caused by pathogenic variants in the fibrillin-1 (FBN1) gene.

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

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

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

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

27 ctive tissue disorder caused by mutations in fibrillin-1 (Fbn1).

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

52 ations that cluster in an internal region of fibrillin-1 called the neonatal region.

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

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

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

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

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

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

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

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

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

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

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

64 Fibrillin-1 deficiency is associated with excess signali

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

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

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

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

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

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

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

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

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

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

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

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

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

78 Circulating fibrillin-1 fragments represent a new potential biomarke

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

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

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

82 Underlying fibrillin-1 gene mutations cause increased transforming

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

84 onnective tissue, caused by mutations in the fibrillin-1 gene.

85 By using a murine model of MFS, fibrillin-1 hypomorphic mgR mice, we found pulmonary emp

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

87 o a potential mechanosensitive mechanism for fibrillin-1 in regulating extracellular transforming gro

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

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

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

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

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 modular glycoprotein that includes 7 la

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

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

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

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

99 Fibrillin-1 microfibril assembly and secreted lysyl oxid

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

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

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

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

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

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

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

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

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

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

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

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

112 Fibrillin-1 mutations are believed to promote abnormal S

113 Fibrillin-1 mutations associated with Marfan syndrome ha

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

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

116 binding integrin to mediate cell adhesion to fibrillin-1 or a disease-causing variant.

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

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

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

120 63 had high levels of FBN1 mRNA and secreted fibrillin-1 protein to form extracellular matrix fibres.

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

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

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

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

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

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

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

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

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

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

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

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

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

134 to be regulated by changes in the ability of fibrillin-1 to mediate integrin binding.

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

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

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

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

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

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

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

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

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

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

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

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

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

148 refore, is that loss of constituents such as fibrillin-1, as in Marfan syndrome, can compromise both

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

171 brils formed by cultured fibroblasts lacking fibrillin-1, which co-localizes with fibronectin and bin

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

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

174 BP4 that connects fibulin-5/tropoelastin and fibrillin-1-binding regions, which have an important rol

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

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

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

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

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

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

181 We observed less densely packed fibrillin-1-rich microfibrils with irregular edges in th

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

183 cules including fibronectin, osteopontin and fibrillin-1.

184 equilibrium encompassing FBN1, which encodes fibrillin-1.

185 functional relationship between ADAMTS10 and fibrillin-1.

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

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

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

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

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

191 ne encoding the extracellular matrix protein fibrillin-1.

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

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

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

195 GD-binding integrins can mediate adhesion to fibrillin-1.

196 ich encodes the extracellular matrix protein fibrillin-1.

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

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

199 um binding extracellular matrix glycoprotein 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 lasmon resonance, binding affinities between fibrillin and all propeptides were determined.

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

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

223 ht into the interactions of tropoelastin and fibrillin and how cross-link formation stabilises the el

224 ated previously that the interaction between fibrillin and tropoelastin, the elastin precursor, incre

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

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

232 The fibrillins are a large family of chloroplast proteins th

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

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

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

236 Fibrillin-based human diseases such as Marfan syndrome a

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

238 Furthermore, tropoelastin and fibrillin can be cross-linked by transglutaminase-2, but

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

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

241 x (ECM) microfibrils that, together with the fibrillins, contributes to microfibril function.

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

243 Fibrillin expression was analyzed by in situ hybridizati

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

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

246 re of gastrulation associated with defective fibrillin fibril assembly.

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

248 f aneurysm patients had detectable levels of fibrillin fragments.

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

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

251 TGFbeta-1, and active TGFbeta-1 did not bind fibrillin in the absence of MAGP-1.

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

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

254 to compare the dynamics of tropoelastin and fibrillin individually as well as in the cross-linked co

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

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

257 Furthermore, when bound to fibrillin, MAGP-1 retained the ability to interact with

258 Fibrillin may thus communicate alterations in matrix to

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

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

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

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

263 rrect formation of elastic fibres requires a fibrillin microfibril scaffold for the deposition of ela

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

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

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

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

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

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

289 Fibrillin-rich microfibrils are the major structural com

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

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

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

293 hin the same domain to differentially affect fibrillin's interactions with distinct RGD-binding integ

294 Fibrillins serve as scaffolds for the assembly of elasti

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 g that occurs due to the covalent linkage of fibrillin to tropoelastin.

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

299 g supports formation of a 1:1 stoichiometric fibrillin-tropoelastin complex.

300 Other fibrillins were located predominantly in the stroma or t

301 components of elastic fibres are elastin and fibrillin, where correct formation of elastic fibres req