ライフサイエンスコーパス: elastic fiber

1 y of tropoelastin monomers into an insoluble elastic fiber.

2 n-5, which absence also leads to compromised elastic fibers.

3 tion of connective tissues, particularly the elastic fibers.

4 g in wild-type mice, before the formation of elastic fibers.

5 amounts of proteins, but structurally normal elastic fibers.

6 mice despite a dramatic reduction of mature elastic fibers.

7 ntrast to the amorphous appearance of normal elastic fibers.

8 and a concomitant disorganization of dermal elastic fibers.

9 bules from the cell membrane onto developing elastic fibers.

10 in required for assembly and organization of elastic fibers.

11 organ involvement, resulting from paucity of elastic fibers.

12 a relatively diminished density of coronary elastic fibers.

13 rmal cysts, and an increase in subpanniculus elastic fibers.

14 and sebaceous glands were the source of the elastic fibers.

15 sponsible for the synthesis of murine dermal elastic fibers.

16 tion of covalent cross-links in collagen and elastic fibers.

17 cles that are patterned on high-aspect-ratio elastic fibers.

18 e consisting of collagen, proteoglycans, and elastic fibers.

19 on microscopy demonstrated markedly abnormal elastic fibers.

20 posits coating the surfaces of frayed dermal elastic fibers.

21 c laminae and the compensatory expression of elastic fibers.

22 oscopy confirmed colocalization of ESMA with elastic fibers.

23 ined with Miller elastic stain had decreased elastic fibers.

24 e, which is indicative of normal, functional elastic fibers.

25 ms microfibrils that act as the template for elastic fibers.

26 onto microfibrils, and cross-linking to form elastic fibers.

27 t these modifications affect the assembly of elastic fibers.

28 Elastin is the predominant component of elastic fibers.

29 species (ROS/RNS) on the assembly of TE into elastic fibers.

30 , oxidatively modified TE was unable to form elastic fibers.

31 Because elastic fiber abnormalities are a central feature of deg

32 Elastic fiber abnormalities in vaginal tissues from youn

33 ability of reproductive tissues to replenish elastic fibers after parturition, leading to pelvic orga

34 ncrease in elastin content was due to dermal elastic fibers, an increase in the size and number of th

35 S (Cat-S), a cysteine protease that degrades elastic fibers and activates the protease-activated rece

36 Progressive fragmentation of elastic fibers and alterations in the regulation of vasc

37 ation of an extracellular matrix enriched in elastic fibers and depleted in hyaluronan, and reduction

38 , Fbln5RGE/RGE mice were able to form intact elastic fibers and did not exhibit POP.

39 ls show enrichment in genes for development, elastic fibers and epigenetic regulation pathways.

40 g proteolytic degradation of existing dermal elastic fibers and for enhancing more efficient elastoge

41 Degradation of elastic fibers and highly expressed CD105 were observed

42 ansfer of fbln5 was sufficient to regenerate elastic fibers and increase elastic fiber-cell connectio

43 lin-5 in facilitating the assembly of normal elastic fibers and inhibiting MMP-9 activity, revealing

44 Akt2-deficient mice showed abnormal elastic fibers and reduced medial thickness in the aorti

45 d cystic medial necrosis (CMN), with loss of elastic fibers and smooth muscle cells.

46 t lysozyme binds to the elastin component of elastic fibers and that this interaction has important b

47 involved in the synthesis and maintenance of elastic fibers and therefore has a strong biological rat

48 lvic organ prolapse (POP) due to compromised elastic fibers and upregulation of matrix metalloproteas

49 eracts with mouse elastin to form functional elastic fibers and when expressed in the elastin haploin

50 and proteoglycans, decreased and fragmented elastic fibers, and cellular disarray without calcificat

51 duced disruption and fragmentation of medial elastic fibers, and decreased Smad2/3 and Erk1/2 activat

52 thickening of the aortic media, disarray of elastic fibers, and increased collagen deposition, toget

53 F and LAM and were associated with collagen, elastic fibers, and smooth muscle cell/myofibroblast-lik

54 Her skin showed markedly underdeveloped elastic fibers, and the extracellular matrix laid down b

55 nesis is re-initiated, but newly synthesized elastic fibers appear abnormal.

56 n of MAGP-2, a component of microfibrils and elastic fibers, appears as an initiating mechanism of in

57 nalysis of the aorta demonstrated fragmented elastic fiber architecture in homozygous mutant null mic

58 The waters of hydration in the elastic fiber are believed to play a critical role in th

59 Although collagen and elastic fibers are among the major structural constituen

60 Elastic fibers are complex structures composed of a trop

61 Elastic fibers are components of the extracellular matri

62 Elastic fibers are composed of the protein elastin and a

63 Elastic fibers are composed of the protein elastin and a

64 sults suggest that synthesis and assembly of elastic fibers are crucial for recovery of pelvic organ

65 Elastic fibers are extracellular structures that provide

66 The elastic fibers are much more uniformly distributed and r

67 These results suggest that fibulin-5 and elastic fibers are not directly involved in short-term w

68 Elastic fibers are typically decreased.

69 Our study suggests that the elastic fibers are under tension and impart an intrinsic

70 soluble elastin, and increased deposition of elastic fibers as compared with empty vector- and biglyc

71 e dual role of fibulin-4 in the formation of elastic fibers as well as terminal differentiation and m

72 l and ultrastructural analyses revealed that elastic fibers assembled normally in the absence of fibu

73 ontent in the vagina suggest that a burst of elastic fiber assembly and cross linking occurs in the v

74 Microfibrils play a role in elastic fiber assembly and serve to link cells to elasti

75 of the C-terminal region of tropoelastin in elastic fiber assembly and suggest tissue-specific diffe

76 ulfation of matrix proteins is important for elastic fiber assembly because inhibition of sulfation w

77 st that fibrillin plays an important role in elastic fiber assembly by binding tropoelastin and perha

78 Inhibition of LOXL1 interfered with elastic fiber assembly by optic nerve head astrocytes in

79 deficiency of microfibrils causes failure of elastic fiber assembly during late fetal development.

80 tivity in cultured fibroblasts and defective elastic fiber assembly in all tissues affected by the di

81 ld be amenable to interventions that restore elastic fiber assembly in the developing lung.

82 Therefore, to clarify the potential role of elastic fiber assembly in the pathogenesis of pelvic org

83 Elastic fiber assembly is a complicated process involvin

84 In an in vitro elastic fiber assembly model, oxidatively modified TE wa

85 egion is conveniently positioned to modulate elastic fiber assembly through association by coacervati

86 alysis showed that MAGP-2 does not stimulate elastic fiber assembly through its RGD motif, suggesting

87 f TE enhanced coacervation, an early step in elastic fiber assembly, but reduced cross-linking and in

88 Nos1; genes whose functions are unrelated to elastic fiber assembly, but whose effects may synergize

89 nteractions with other proteins required for elastic fiber assembly, including fibulin-4, fibulin-5,

90 matricellular protein that is essential for elastic fiber assembly, regulated the activity of MMP-9

91 To determine whether MAGP-2 regulates elastic fiber assembly, we used an in vitro model featur

92 further elucidate the molecular mechanism of elastic fiber assembly.

93 ptor binding does not mediate MAGP-2-induced elastic fiber assembly.

94 fiber onset, suggesting that MAGP-2 mediates elastic fiber assembly.

95 tic fibers without a clearly defined role in elastic fiber assembly.

96 of normal elastoblasts or specifically alter elastic fiber assembly.

97 at microfibril-associated MAGP-2 facilitates elastic fiber assembly.

98 ic elastin and the matrix scaffold to aid in elastic fiber assembly.

99 elastogenesis do not mediate MAGP-2-induced elastic fiber assembly.

100 poelastin, indicating that MAGP-2 stimulates elastic fiber assembly.

101 ue, stable in vitro system in which to study elastic fiber assembly.

102 tential to serve as a cell culture model for elastic fiber assembly.

103 he importance of this region in facilitating elastic fiber assembly.

104 of the bridge region, which is critical for elastic fiber assembly.

105 nd these interactions play critical roles in elastic fiber assembly.

106 recently found that oxidation of TE prevents elastic fiber assembly.

107 ely contributing to its nonredundant role in elastic fiber assembly.

108 The expression of tropoelastin and other elastic fiber-associated genes was not significantly mod

109 in-1, suggesting that microfibrils and other elastic fiber-associated proteins known to regulate elas

110 inhibitor that exerts a protective effect on elastic fibers at sites of tissue injury.

111 The lack of elastic fibers at the hypodermal muscle-connective tissu

112 that fibulin-4-/- mice do not develop intact elastic fibers but contain irregular elastin aggregates.

113 Both mutants failed to be incorporated into elastic fibers by transfected rat lung fibroblasts.

114 he functional relationship between ABCC6 and elastic fiber calcification is unknown.

115 f abnormalities in the vessel wall including elastic fiber calcification, excessive deposition of mat

116 monstrate how a simple model for networks of elastic fibers can quantitatively account for the mechan

117 nt to regenerate elastic fibers and increase elastic fiber-cell connections in vivo.

118 ment membrane, cell adhesive structures, and elastic fibers characterized by the presence of a unique

119 Protein and mRNA expression of major elastic fiber components (elastin, fibrillin-1, fibulin-

120 ay contribute to the abnormal aggregation of elastic fiber components into characteristic PEX fibrils

121 Major elastic fiber components were present in the skin of fbl

122 ar locations and to co-localize with various elastic fiber components.

123 zed molecular interactions between MFAP4 and elastic fiber components.

124 sed on experimentally measured alteration in elastic fiber composition, alveolar geometry and surfact

125 Extracellular elastic fibers confer resilience and flexibility to tiss

126 Elastic fibers consist of two morphologically distinct c

127 cant coordinated downregulation of LOXL1 and elastic fiber constituents on mRNA and protein level.

128 Elastic fibers contribute to the structural support of t

129 f elastin globules to fuse into a continuous elastic fiber core.

130 CB-EGF domain, however, was unable to rescue elastic fiber defects.

131 re associated with matrix microstructure and elastic fiber deficiencies and may influence the hydrati

132 ed WSS correspond with ECM dysregulation and elastic fiber degeneration in the ascending aorta of BAV

133 te features of plaque instability, including elastic fiber degradation and fibrous cap thinning, by h

134 ypocomplementemia and propose a mechanism of elastic fiber degradation in the skin of this patient wi

135 on has important biological consequences for elastic fiber degradation.

136 e significantly attenuated POP by increasing elastic fiber density and improving collagen fibrils.

137 lteration in lung recruitment and diminished elastic fiber density were shown predictive of mechanica

138 ate difference from the pubertal controls in elastic fiber density.

139 These results show that a systemic defect in elastic fiber deposition affects Bruch's membrane integr

140 However, the mechanism of elastic fiber development and assembly is poorly underst

141 is an elastin-binding protein essential for elastic fiber development in vivo, and it has recently b

142 elastin and fibrillin-1 leading to impaired elastic fiber development.

143 pertension, all of which are associated with elastic fiber disease.

144 g Fbln3(-[supi]/-) mice progressed to severe elastic fiber disruption with age, and vaginal matrix me

145 so show that infliximab treatment attenuated elastic fiber disruption, macrophage infiltration, and M

146 teins associated with the basement membrane, elastic fibers (emilin-1), and other extracellular prote

147 ughout the stretching process, and prominent elastic fiber engagement is observed up to 20% strain af

148 influences the assembly of ECM and controls elastic fiber fibrillogenesis, which is of fundamental i

149 These data demonstrate the importance of elastic fibers for maintaining structural and functional

150 l recessive cutis laxa and marked defects in elastic fiber formation amplifies previous observations

151 We conclude that fibulin-4 is necessary for elastic fiber formation and connective tissue developmen

152 brillin microfibrils have essential roles in elastic fiber formation and elastic tissue homeostasis,

153 -2 is not required for mouse development and elastic fiber formation and suggest possible functional

154 e hypothesis that fibulin-5 is necessary for elastic fiber formation by facilitating the deposition o

155 s have been shown to play essential roles in elastic fiber formation during development.

156 ucidate the molecular mechanisms of impaired elastic fiber formation in recessive cutis laxa, we have

157 A mutant protein additionally showed reduced elastic fiber formation upon addition to human retinal p

158 sion as a marker of tissues participating in elastic fiber formation.

159 rdiovascular compromise, is due to defective elastic fiber formation.

160 lin-1 reduces tissue homeostasis rather than elastic fiber formation.

161 The principal protein component of the elastic fiber found in elastic tissues is elastin, an am

162 a of AAA, where it is also bound to residual elastic fiber fragments.

163 Synthesis of abnormal TE may interfere with elastic fiber function through a dominant-negative or a

164 amount of mature elastin within 3 wk and the elastic fibers had similar orientation as those in nativ

165 hibitors in association with changes in lung elastic fibers has been implicated in the pathogenesis o

166 Here we show that a failure to maintain elastic fiber homeostasis in mice causes pelvic floor di

167 aracterized in Fbln5-/- mice, and changes in elastic fiber homeostasis in the mouse vagina during pre

168 t after vaginal delivery and that disordered elastic fiber homeostasis is a primary event in the path

169 ings raise the possibility that a failure of elastic fiber homeostasis, either due to genetic predisp

170 primary disturbance in LOXL1 regulation and elastic fiber homeostasis, possibly rendering pseudoexfo

171 Elastic fiber in all aortas showed little or no variatio

172 ceptors (Flg, Bek) and measured collagen and elastic fiber in lung sections from IPF (n = 14), LAM (n

173 in is the principal protein component of the elastic fiber in vertebrate tissue.

174 microlithiasis without the calcification of elastic fibers in arterial walls that is characteristic

175 owed inflammation followed by destruction of elastic fibers in both the skin and the aorta.

176 racts with tropoelastin and colocalizes with elastic fibers in culture.

177 l/L) selectively stimulates formation of new elastic fibers in cultures of human aortic smooth muscle

178 e disorder mainly characterized by calcified elastic fibers in cutaneous, ocular, and vascular tissue

179 e of fibulin-5 and the effect of the loss of elastic fibers in dermal wound healing.

180 late onset and progressive mineralization of elastic fibers in dermal, ocular, and cardiovascular tis

181 ical analysis revealed a marked reduction of elastic fibers in fascia, a thin layer of connective tis

182 has been shown to be associated with damaged elastic fibers in many tissues and organs.

183 n and fibrillin-1, in vitro and localizes to elastic fibers in many tissues in vivo.

184 y that LTBP-2 plays a structural role within elastic fibers in most cases.

185 ntrast agent (ESMA) for in vivo targeting of elastic fibers in myocardial infarction (MI) and postinf

186 The distribution of elastic fibers in ONH tissue was investigated by immunof

187 te to the abnormal structure and function of elastic fibers in pathological conditions.

188 helpful in predicting loss of aortic medial elastic fibers in patients with ascending aortic aneurys

189 It is associated with elastic fibers in several tissues and is believed to ser

190 c degeneration and calcification of vascular elastic fibers in the absence of aneurysm formation and

191 r areas stained positive for the presence of elastic fibers in the extracellular matrix.

192 ic fiber assembly and serve to link cells to elastic fibers in the extracellular matrix.

193 transfected PE cells showed the presence of elastic fibers in the matrix.

194 Most engineered vessels lack elastic fibers in the medial layer and those present are

195 extracellular matrix with disorganization of elastic fibers in the optic nerve head (ONH).

196 Electron microscopy revealed aberrant elastic fibers in the papillary dermis of photoaged fore

197 n mice, mutant elastin was incorporated into elastic fibers in the skin and lung with adverse effects

198 By electron microscopy, elastic fibers in the skin of this patient showed a fail

199 haracterized by progressive calcification of elastic fibers in the skin, eyes, and the cardiovascular

200 tains of en face sections suggested that the elastic fibers in the upper dermis were exclusively deri

201 oxidase-like 1 (LOXL1) do not deposit normal elastic fibers in the uterine tract post partum and deve

202 eased vaginal protease activity and abnormal elastic fibers in the vaginal wall are important compone

203 The increased elastic fibers in Tsk hypodermal connective tissue was a

204 teins associated with basement membranes and elastic fibers in vertebrates.

205 sera interfered with the normal assembly of elastic fibers in vitro and suggested that PXE is a prim

206 d salt is a critical step in the assembly of elastic fibers in vivo, preceding chemical cross-linking

207 In response to UV irradiation, the elastic fibers increased in number and size, wrapping ar

208 ponents C1q and C3 on the surfaces of dermal elastic fibers, indicating complement fixation by the de

209 The lack of elastic fiber involvement establishes the uniqueness of

210 Most notably, there was no dermal elastic fiber involvement, limited cutaneous and systemi

211 Failure to maintain elastic fibers is explained by a theory of antielastase-

212 In Tsk mice, a prominent elastic fiber layer found normally at the interface betw

213 MAGP-2 overexpression dramatically increased elastic fibers levels, independently of extracellular le

214 The density profile of an elastic fiber like DNA will change in space and time as

215 Ascending aortic medial elastic fiber loss (EFL) (graded 0 to 4+) was zero or 1+

216 s characterized by fragmentation and loss of elastic fibers, loss of smooth muscle cells, and interst

217 microfibril-associated MAGP-2 may stimulate elastic fiber macroassembly by targeting the release of

218 action and cell motility, two key factors in elastic fiber macroassembly, microfibril-associated MAGP

219 in polymer deposition is a crucial aspect of elastic fiber maintenance and is dependent on LOXL1, whi

220 tudy investigated the influence of defective elastic fiber maintenance in the development of laser-in

221 dation of intercellular matrix, particularly elastic fibers, may play a key role in the pathogenesis

222 fibulin-3, -4, and -5, are components of the elastic fiber/microfibril system and are implicated in t

223 nounced (ultra)structural alterations of the elastic fiber network in the laminar beams of pseudoexfo

224 ontribution of the extracellular microfibril-elastic fiber network to vertebrate organogenesis, we ge

225 crofibrils, key structural components of the elastic fiber network, in photoaged and photoprotected s

226 ain, rVN, showed that rVN deposited onto the elastic fiber network.

227 amina cribrosa in association with a complex elastic fiber network.

228 dermal matrix components, with the aberrant elastic fibers no longer apparent.

229 Elastin, the principal component of the elastic fiber of the extracellular matrix, imparts to ve

230 k fibrillin-1 leads to marked alterations in elastic fibers of the hypodermis of Tsk animals.

231 significant increase in the thickness of the elastic fibers of the trabeculum ciliare covering the an

232 exhibits developmental expression peaking at elastic fiber onset, suggesting that MAGP-2 mediates ela

233 by impairing the homeostasis of established elastic fibers, or by a combination of both mechanisms.

234 l-derived inflammation resulted in disrupted elastic fiber organization and down-regulation of elasti

235 ofibrils and tropoelastin involved in proper elastic fiber organization.

236 scopy confirmed an upregulation of LOXL1 and elastic fiber proteins and their assembly into extracell

237 Therefore, we compared the deposition of elastic fiber proteins in cultures of fibroblasts derive

238 nockout mice or mice with knockouts of other elastic fiber proteins, implying that LTBP-2 performs a

239 sion of transforming growth factor-beta1 and elastic fiber proteins.

240 Elastic fibers provide tissues with elasticity which is

241 Elongation of the elastic fibers results in a shift of the reflection of o

242 his may be correlated with thickening of the elastic fiber sheath in the CM tips in addition to other

243 The surfaces of disrupted elastic fibers showed IR for MMP-2 and MMP-9.

244 and have important roles in microfibril and elastic fiber structure, homeostasis, and vascular devel

245 eraction of ELN and FBLN5 alleles results in elastic fibers susceptible to inflammatory destruction.

246 Fibulin-5 is crucial for normal elastic fiber synthesis in the vaginal wall; more than 9

247 oma is a complex, late-onset disorder of the elastic fiber system.

248 ed with TA or EA, deposit significantly more elastic fibers than untreated control cultures despite t

249 uding unusual atrophic patches with abnormal elastic fibers that can sometimes be the first noted sig

250 acellular matrix protein that assembles into elastic fibers that provide elasticity and strength to v

251 Female reproductive organs are rich in elastic fibers that turn over slowly in most adult tissu

252 ressive proteolytic degradation of cutaneous elastic fibers, that cannot be adequately replaced or re

253 n addition to showing reduced and fragmented elastic fibers, the histopathological hallmark of cutis

254 stin-binding protein that scaffolds cells to elastic fibers, thereby preventing elastinopathy in the

255 ely, cell processes associated with zones of elastic fiber thinning and fragmentation.

256 These mice also formed disorganized elastic fibers throughout hypodermal connective tissue a

257 MAGP-2 can function outside of their role in elastic fibers to activate a cellular signaling pathway.

258 e specimens demonstrating differentiation of elastic fibers, triglycerides, collagen, myelin, cellula

259 n-2 interacts with two major constituents of elastic fibers, tropoelastin and fibrillin-1, in vitro a

260 Collagen and elastic fiber turnover was greater in TIC, as was the my

261 We study the enzymatic degradation of an elastic fiber under tension using an anisotropic random-

262 we investigate the long-term maintenance of elastic fibers under tension combined with diffusion of

263 Loss of elastic fibers underlies connective tissue aging and imp

264 the ADM, while retention of the transplanted elastic fibers was apparent.

265 in essential for the postnatal deposition of elastic fibers, was highly expressed and regulated in th

266 Histologically, elastic fibers were more abundant in group A than in gro

267 ial calcium deposits, associated mainly with elastic fibers, were persistently accompanied by elastin

268 uscle cells deposited abnormal aggregates of elastic fibers when maintained in the presence of serum

269 inking results in 2.5x stiffer and 1.5x more elastic fibers, whereas full cross-linking results in 3.

270 haracterized by progressive calcification of elastic fibers with a pathognomonic histological appeara

271 nt can bind cells normally, it does not form elastic fibers with human dermal fibroblasts and forms f

272 sed of loose connective tissue (collagen and elastic fibers) with focal areas of myxoid stroma, with

273 revealed elongation and increased density of elastic fibers, with an increase in dermal collagen and

274 4) is an extracellular glycoprotein found in elastic fibers without a clearly defined role in elastic

275 h type 1 diabetes, in which induction of new elastic fibers would mechanically stabilize the developi