ライフサイエンスコーパス: desmosomal

1 is an armadillo family protein critical for desmosomal adhesion and epidermal integrity.

2 occurring mutant mice revealed disruption of desmosomal adhesion and perturbations in keratinocyte be

3 Using 1023 as a tool, we identified desmosomal adhesion as a novel target of AHR signaling a

4 This assay relates directly to desmosomal adhesion because it involves splitting of the

5 We demonstrate that modulation of desmosomal adhesion by PKC can regulate migration of tro

6 s provide novel mechanisms for regulation of desmosomal adhesion by RhoA- and PKC-mediated adducin ph

7 This study beautifully demonstrates that desmosomal adhesion can be modulated by the molecular in

8 which autoantibodies against proteins of the desmosomal adhesion complex perturb desmosomal function,

9 ral disease.It is believed that mutations in desmosomal adhesion complex protein plakophilin 2 (PKP2)

10 ance of this protein in the stabilization of desmosomal adhesion in terminally differentiating kerati

11 Desmosomal adhesion is Ca(2+) dependent, and reduction o

12 ratinocytes, and decreased the levels of the desmosomal adhesion molecule desmoglein (Dsg)3 by reduci

13 usly, we have reported that depletion of the desmosomal adhesion molecule desmoglein (Dsg)3 induced b

14 in which antibodies are directed against the desmosomal adhesion molecule Dsg3, resulting in severe m

15 characterized by autoantibodies against the desmosomal adhesion protein desmoglein (Dsg) 3.

16 isease associated with autoantibodies to the desmosomal adhesion protein, desmoglein 3.

17 ranes caused by pathogenic autoantibodies to desmosomal adhesion proteins desmoglein 3 (Dsg3) and Dsg

18 Wounding results in weakening of desmosomal adhesion to a calcium-dependent state, presum

19 s well-known role in mediating intercellular desmosomal adhesion, Dsg2 regulates mitogenic signaling

20 gesting a direct effect of PKC signalling on desmosomal adhesion.

21 is required for establishing and maintaining desmosomal adhesion.

22 for Perp in promoting the stable assembly of desmosomal adhesive complexes.

23 face in patient tissue is due to compromised desmosomal adhesive function.

24 dhesion because it involves splitting of the desmosomal adhesive material.

25 ility that internalization and regulation of desmosomal and classic cadherin function can be uncouple

26 a key linkage in protein chains that connect desmosomal and classical cadherins to the cytoskeleton.

27 the notion of a molecular crosstalk between desmosomal and gap junction proteins.

28 cing data were interrogated for mutations in desmosomal and other skin structural genes, followed by

29 the production of autoantibodies against the desmosomal antigen desmoglein-1 (Dsg1).

30 target the hemidesmosomal antigen BP180 and desmosomal antigens Dsg1 and Dsg3, respectively.

31 study, we provide evidence that loss of the desmosomal armadillo protein Plakophilin-2 (PKP2) in car

32 The architectures of native desmosomal assemblies have been visualized by cryo-elect

33 sylated glycoproteins followed by binding to desmosomal-associated JAM-C are key elements of the tran

34 se tissues during embryogenesis, and certain desmosomal blistering diseases such as pemphigus vulgari

35 We identify the desmosomal BM remodelling serine protease Hepsin as a ke

36 s indicated that junctional incorporation of desmosomal, but not adherens junction, components was im

37 report a novel role of Gal3 in stabilizing a desmosomal cadherin and intercellular adhesion in intest

38 smocollin 2 increased 1.7-2.0-fold, and both desmosomal cadherin and plaque components were recruited

39 suggest that desmocollin-1 can function as a desmosomal cadherin both in basal and suprabasal cells.

40 g2 processing, supporting the idea that this desmosomal cadherin can be regulated by multiple ADAM fa

41 ferentiation via proteolytic cleavage of the desmosomal cadherin component desmoglein 1 (Dsg1).

42 The desmosomal cadherin desmocollin (Dsc)1 is expressed in u

43 The desmosomal cadherin desmoglein 2 (Dsg2) localizes to the

44 re the structure of the entire ectodomain of desmosomal cadherin desmoglein 2 (Dsg2), using a combina

45 lgaris (PV), autoantibodies (IgG) target the desmosomal cadherin desmoglein 3 (Dsg3) and compromise k

46 mporal dynamics of order and disorder of the desmosomal cadherin desmoglein 3 (Dsg3) in living cells.

47 ted a central role for downregulation of the desmosomal cadherin desmoglein 3 (DSG3) in the pathogene

48 IgG autoantibodies directed against the desmosomal cadherin desmoglein 3 (Dsg3), the major autoa

49 dies against the extracellular domain of the desmosomal cadherin desmoglein 3 cause potentially fatal

50 utoantibodies (IgG) are directed against the desmosomal cadherin desmoglein 3.

51 we have focused on the palmitoylation of the desmosomal cadherin desmoglein-2 (Dsg2) and characterize

52 vulgaris (PV) pathogenic antibodies bind the desmosomal cadherin desmoglein-3 (dsg3), causing epiderm

53 er caused by antibodies directed against the desmosomal cadherin desmoglein-3 (Dsg3).

54 The autoantibodies generated target the desmosomal cadherin desmoglein-3 (Dsg3).

55 in turn promotes differentiation through the desmosomal cadherin Dsg1.

56 lds but, with the exception of classical and desmosomal cadherin EC1 domains, most of them do not app

57 n desmoglein 4 (DSG4), a novel member of the desmosomal cadherin family that is expressed in the hair

58 omes contain multiple representatives of the desmosomal cadherin family, which includes three desmogl

59 clusters on chromosomes 12q and 17q, and the desmosomal cadherin gene cluster on chromosome 18q.

60 clusters on chromosomes 12q and 17q and the desmosomal cadherin gene cluster on chromosome 18q.

61 els of 2 genes as the primary genes: DSG2, a desmosomal cadherin involved in Wnt/beta-catenin signali

62 c deletion of desmocollin 3, the other major desmosomal cadherin isoform expressed in the basal epide

63 Rules governing the assembly of desmosomal cadherin isoforms into desmosomes of differen

64 -terminal fragment of desmoglein 2 (Dsg2), a desmosomal cadherin often overexpressed in malignancies.

65 We previously showed that a desmosomal cadherin promotes keratinocyte differentiatio

66 Desmoglein-2 (Dsg2) is a desmosomal cadherin that is aberrantly expressed in huma

67 Desmoglein 1 (Dsg1) is a desmosomal cadherin that is essential to epidermal integ

68 ly upon the up-regulation of desmoglein 1, a desmosomal cadherin that maintains the integrity and dif

69 Differential regulation of desmosomal cadherin transport could provide a mechanism

70 role for endocytic trafficking in regulating desmosomal cadherin turnover and function and raise the

71 FR inhibition results in accumulation of the desmosomal cadherin, desmoglein 2 (Dsg2), at cell-cell i

72 recognizing several proteins, including the desmosomal cadherin, desmoglein 3.

73 both a classical cadherin, P-cadherin, and a desmosomal cadherin, desmoglein 3.

74 The desmosomal cadherin, desmoglein-1 (DSG1), promotes kerat

75 dy we show that loss of the other intestinal desmosomal cadherin, desmoglein-2 (Dsg2) that pairs with

76 We show that Dsg2 but not another desmosomal cadherin, Dsc2, is cleaved by cysteine protea

77 ring disease in which antibodies against the desmosomal cadherin, DSG3 (desmoglein-3), cause acanthol

78 alloproteinase-dependent proteolysis of this desmosomal cadherin.

79 f possible synergism between a classical and desmosomal cadherin.

80 Using purified proteins, we show that desmosomal cadherins and alpha-catenin compete directly

81 these diseases, autoantibodies against other desmosomal cadherins and E-cadherin may also be present.

82 c areas possess autoantibodies against other desmosomal cadherins and E-cadherin.

83 histone deacetylase inhibition up-regulates desmosomal cadherins and prevents the loss of adhesion i

84 ll adhesion by compromising the link between desmosomal cadherins and the intermediate filament cytos

85 ing list of human mutations that target both desmosomal cadherins and their associated cytoskeletal a

86 atable connection between both classical and desmosomal cadherins and their respective cytoskeletal l

87 he intermediate filament cytoskeleton to the desmosomal cadherins and thereby confers structural stab

88 The desmosomal cadherins are calcium-dependent transmembrane

89 Characteristic patterns of desmosomal cadherins are tightly regulated and distinct

90 Desmosomal cadherins are transmembrane adhesion molecule

91 etween adjacent cells, this study implicates desmosomal cadherins as key components of a signaling ax

92 Modulation of the palmitoylation status of desmosomal cadherins can affect desmosome dynamics.

93 The Dsg subclass of desmosomal cadherins contains a C-terminal unique region

94 caused by autoantibodies primarily targeting desmosomal cadherins desmoglein 3 (DSG3) and DSG1, leadi

95 ogether, these data demonstrate that partner desmosomal cadherins Dsg2 and Dsc2 play opposing roles i

96 e potential role of differentiation-specific desmosomal cadherins during apoptosis has not been exami

97 This fit suggests an arrangement in which desmosomal cadherins form trans interactions but are too

98 uses a reduction in the levels of endogenous desmosomal cadherins in a dose-dependent manner, leading

99 : type 1 cadherins in adherens junctions and desmosomal cadherins in desmosomes.

100 othesized that the arrangement, or order, of desmosomal cadherins in the intercellular space is criti

101 Desmosomal cadherins mediate cell-cell adhesion in epith

102 ve strength is the level and organization of desmosomal cadherins on the cell surface.

103 and highlight a novel mechanism by which the desmosomal cadherins regulate beta-catenin signaling.

104 punctate structures made up of transmembrane desmosomal cadherins termed desmoglein-2 (Dsg2) and desm

105 Desmogleins are desmosomal cadherins that mediate cell-cell adhesion.

106 cyte adhesion in linking the transmembranous desmosomal cadherins to the cytoplasmic keratin filament

107 integral part of desmosomes, where it links desmosomal cadherins to the intermediate filaments.

108 Our observations illustrate a new mechanism desmosomal cadherins use to control their surface levels

109 igus is caused by IgG autoantibodies against desmosomal cadherins, but the precise mechanisms are in

110 Suprabasal layers upregulated desmosomal cadherins, but without classical cadherins, t

111 dillo repeat region reduces the affinity for desmosomal cadherins, calorimetric measurements show no

112 The desmosomal cadherins, comprising the desmogleins and des

113 wn that one of the two intestinal epithelial desmosomal cadherins, desmocollin-2 (Dsc2) loss promotes

114 The intercellular interactions of the desmosomal cadherins, desmoglein and desmocollin, are re

115 Desmosomal cadherins, desmogleins (Dsgs) and desmocollin

116 The desmosomal cadherins, desmogleins (Dsgs) and desmocollin

117 e it binds to the cytoplasmic domains of the desmosomal cadherins, desmogleins and desmocollins.

118 The desmosomal cadherins, desmogleins, and desmocollins medi

119 n desmosome assembly, interactions among the desmosomal cadherins, desmoplakin, and the armadillo fam

120 , which are characterized by the presence of desmosomal cadherins, known as desmogleins and desmocoll

121 atively normal intercellular distribution of desmosomal cadherins, their cytoplasmic plaques are spar

122 somes mediate intercellular adhesion through desmosomal cadherins, which interface with plakoglobin (

123 keleton, but only gamma-catenin binds to the desmosomal cadherins, which links them to intermediate f

124 ness depends on the organised arrangement of desmosomal cadherins.

125 nents, in which they link desmoplakin to the desmosomal cadherins.

126 nity to desmoglein 3, desmocollin 3, or both desmosomal cadherins.

127 ent (IF)-binding protein desmoplakin (DP) to desmosomal cadherins.

128 attention has been paid to the importance of desmosomal cadherins.

129 ensus sequence not conserved among the other desmosomal cadherins.

130 , including both acetylcholine receptors and desmosomal cadherins.

131 not dependent on the equimolar expression of desmosomal cadherins.

132 Desmocollin 1 (Dsc1) is part of a desmosomal cell adhesion receptor formed in terminally d

133 The importance of desmosomal cell adhesion to human health is evidenced by

134 In addition to their role in desmosomal cell-cell adhesion, Pkps also localize to the

135 P, and KRT4, as well as DSG1, a component of desmosomal cell-cell junctions.

136 and the stratum corneum was detached through desmosomal cleavage.

137 ) serves to anchor intermediate filaments in desmosomal complexes.

138 teolysis of corneodesmosin, an extracellular desmosomal component.

139 Expression of desmosomal components Dsp, Dsg-1a, and Dsg-1b was downre

140 (ARVC) is a phenotype caused by mutations in desmosomal components in approximately 50% of patients,

141 Immunofluorescence (IF) for desmosomal components on cryostat sections of fresh epit

142 Subsequently, Dsg3 and other desmosomal components rearrange into linear arrays that

143 lin 2 interacts with a broader repertoire of desmosomal components than plakophilin 1 and provide new

144 yte adhesion, the fate of PV IgG and various desmosomal components was monitored in primary human ker

145 Desmosomal components were downregulated, consistent wit

146 (PG), is involved in coupling transmembrane desmosomal components with IFs.

147 efects, characterized by decreased levels of desmosomal components, decreased attachment of keratin f

148 ion between the Cops3 subunit of the CSN and desmosomal components, Desmoglein1 (Dsg1) and Desmoplaki

149 ophilin 2 can interact directly with several desmosomal components, including desmoplakin, plakoglobi

150 uscle lacks desmosomes, it contains multiple desmosomal components, including plakoglobin.

151 The obligate desmosomal constituent, plakoglobin (PG), is involved in

152 tance of the classic mechanical functions of desmosomal constituents is underscored by pathologies re

153 ape transitions are accompanied by a loss of desmosomal contacts, an increase in cell motility, and a

154 ted by pathogenic autoantibodies against the desmosomal core glycoprotein desmoglein-1 (Dsg1).

155 order translocation of desmoplakin (DP), the desmosomal cytolinker protein necessary for intermediate

156 et al. describe how enhanced expression of a desmosomal cytoplasmic plaque protein, plakophilin-1, pr

157 Lis1 ablation also causes desmosomal defects, characterized by decreased levels of

158 of the cells to apidogenic stimuli augmented desmosomal distortion and lipid accumulation.

159 tinocytes by enhancing both the depletion of desmosomal DSG3 and intercellular adhesion defects.

160 eratinocytes, p38 knockdown prevents loss of desmosomal Dsg3 by PV mAbs, and exogenous p38 activation

161 ed keratin filament retraction, and promoted desmosomal DSG3 oligomerization.

162 gests that the phenotype is a consequence of desmosomal fragility associated with premature proteolys

163 d desmin, iASPP is an important regulator of desmosomal function both in vitro and in vivo.

164 s of the desmosomal adhesion complex perturb desmosomal function, leading to intercellular adhesion d

165 n of whether basal desmocollin-1 could alter desmosomal functions and compromise keratinocyte prolife

166 ROCK activity and its downstream effects on desmosomal gene expression.

167 ase is most often caused by mutations in the desmosomal gene for plakophilin-2 (PKP2), which is expre

168 The overall study population included 134 desmosomal gene mutation carriers (68 men; median age 36

169 gosity was identified in 16% of ARVC-causing desmosomal gene mutation carriers and was a powerful ris

170 red thirteen patients (84%) carried a single desmosomal gene mutation in desmoplakin (n=44; 39%), pla

171 Multiple desmosomal gene mutations and male sex were independent

172 subjects with ARVC; of these samples, 8 had desmosomal gene mutations.

173 An additional desmosomal gene variant was found in 10% and was associa

174 mic events and sudden cardiac death (SCD) in desmosomal gene-related ARVC.

175 need to determine the prevalence of CNVs in desmosomal genes and to evaluate disease penetrance by c

176 f 160 AC genotype-negative probands for 5 AC desmosomal genes by conventional mutation screening unde

177 he use of comprehensive genetic screening of desmosomal genes for arrhythmic risk stratification in A

178 In ARVC, 5 causative desmosomal genes have been identified, but because only

179 osting PKP2 variants were screened for other desmosomal genes mutations; second variants (digenic het

180 Heterozygous mutations in non-PKP 2 desmosomal genes occurred in 14 of 198 subjects (7%), in

181 isease of cell adhesion because mutations in desmosomal genes, desmoplakin and plakoglobin, have been

182 s negative for pathogenic point mutations in desmosomal genes, highlighting the potential of CNVs ana

183 o-event analysis, and was stratified by sex, desmosomal genes, mutation types, and genotype complexit

184 ons but not in those with mutations in other desmosomal genes.

185 rtance of heart examination of patients with desmosomal genodermatoses.

186 inant subclasses of autoantibodies against a desmosomal glycoprotein, desmoglein-1 (Dsg1).

187 Of the desmosomal glycoproteins, desmocollin 2 (Dsc2) and desmo

188 elia, cornea expresses only a single pair of desmosomal glycoproteins, Dsc2 and Dsg2.

189 ase in extracellular space and a loss of the desmosomal intercellular midline.

190 claudin-1, and claudin-4, as well as that of desmosomal junction proteins corneodesmosin and desmogle

191 orms result from mutations in genes encoding desmosomal junction proteins.

192 ramatic rearrangement of tight junctions and desmosomal junctions in apoptotic monolayers.

193 te that polycystin-1 is a novel component of desmosomal junctions of epithelial cells.

194 hrough cadherin junctions, both adherens and desmosomal junctions, strengthened by association with c

195 dogenous polycystin-1 distributed with IF at desmosomal junctions.

196 Desmosomal localization of JAM-C was further confirmed b

197 an desmocollin-1 colocalized with endogenous desmosomal marker proteins, indicating efficient incorpo

198 atinocyte cell surfaces and colocalized with desmosomal markers.

199 e classes of autoantibodies directed against desmosomal, mitochondrial, and other keratinocyte self-a

200 gainst desmoglein 1, a 160 kDa transmembrane desmosomal molecule expressed in keratinocytes.

201 ARVC desmosomal mutation carriers (n = 84) were evaluated by

202 Forty-two pathogenic desmosomal mutation carriers with definite ARVD/C based

203 dysplasia/cardiomyopathy (ARVD/C)-associated desmosomal mutation carriers without histories of sustai

204 ncrease the risk of VT/VF, HF, and ARVD/C in desmosomal mutation carriers.

205 +/- 17 years; 18 males) family members of 12 desmosomal mutation-carrying ARVD/C probands underwent g

206 mosome, current knowledge on the relation of desmosomal mutations and disease phenotypes, and an over

207 The discovery of desmosomal mutations associated with ARVD/C has led rese

208 he structural and functional consequences of desmosomal mutations can now begin to be understood at m

209 junctional cytoarchitecture in subjects with desmosomal mutations confirms that ARVC is a disease of

210 thmogenic cardiomyopathy patients with known desmosomal mutations when compared with controls.

211 /cardiomyopathy (ARVD/C) among patients with desmosomal mutations.

212 and nucleus, with gamma-catenin, one of its desmosomal partners, and with beta-catenin and TCF7L2, e

213 the ARVC-CMs that displayed the more severe desmosomal pathology.

214 e provide new evidence that mutations in the desmosomal plakophilin-2 gene can cause ARVC.

215 n association of the 195-kD protein with the desmosomal plaque and with keratin filaments in the diff

216 convergence upon the cytoplasmic face of the desmosomal plaque as well as in the nucleus.

217 peat-containing proteins first identified as desmosomal plaque components, in which they link desmopl

218 th minimal impact on distribution of Dsc2 or desmosomal plaque components.

219 that manipulating the expression of a single desmosomal plaque protein can block the pathogenic effec

220 s, we find that PKP-1 clusters Dsg3 with the desmosomal plaque protein desmoplakin in a manner depend

221 The constitutive desmosomal plaque protein desmoplakin plays a vital part

222 of a human mutation in the gene encoding the desmosomal plaque protein, desmoplakin, has been describ

223 ane receptor, which does not bind the common desmosomal plaque proteins plakoglobin and plakophilin 1

224 containing proteins, initially identified as desmosomal plaque proteins that have subsequently been s

225 c1a variant is essential for assembly of the desmosomal plaque, a structure that connects desmosomes

226 Desmoplakin, a constitutive component of the desmosomal plaque, is the most abundant protein present

227 onents to form an adhesive interface and the desmosomal plaque.

228 and failed translocation of loricrin to the desmosomal plaques.

229 In this paper, we report that the desmosomal protein desmoplakin (DP) is not essential for

230 ition to binding intermediate filaments, the desmosomal protein desmoplakin (DP) regulates microtubul

231 binding protein end-binding 1 (EB1) and the desmosomal protein desmoplakin (DP), and demonstrate tha

232 ent protein while concomitantly deleting the desmosomal protein desmoplakin in cardiac myocyte lineag

233 emonstrate that cardiac-specific loss of the desmosomal protein desmoplakin is sufficient to cause nu

234 Signal for the desmosomal protein desmoplakin was reduced in buccal muc

235 y, due to a thinned epidermis with decreased desmosomal protein expression and incomplete biochemical

236 ic cardiomyopathy are caused by mutations in desmosomal protein genes has galvanized interest in the

237 This work identifies a novel function for a desmosomal protein in regulating microtubules that affec

238 primary heart muscle disorder resulting from desmosomal protein mutations.

239 ression leads to nuclear localization of the desmosomal protein plakoglobin and a 2-fold reduction in

240 Immunoreactive signals for the desmosomal protein plakoglobin and the major cardiac gap

241 ism, we generated transgenic mice expressing desmosomal protein plakoglobin in myocyte lineages.

242 ed reduced densities of PKP2, the associated desmosomal protein plakoglobin, and the gap-junction pro

243 Signal for the desmosomal protein plakophilin-1 was reduced in buccal m

244 o the adherens junction protein p120 and the desmosomal protein plakophilin-1.

245 embrane protein, structurally interacts with desmosomal protein plakophilin-2 (PKP2), basal ES protei

246 udy reported mutations in PKP2, encoding the desmosomal protein plakophilin-2, associated with ARVD/C

247 very of mutant human PKP2, which encodes the desmosomal protein plakophilin-2.

248 Desmoplakin is a cytoplasmic desmosomal protein that plays a vital role in normal int

249 he proteins of the tight junction (cadherin, desmosomal protein) by quantitative immunoperoxidase and

250 rtance of Perp, a newly discovered tetraspan desmosomal protein, in PV.

251 Desmoglein-1 (DSG1), a desmosomal protein, maintains the structure of epidermis

252 Mutations in 6 genes, including 4 encoding desmosomal proteins (Junctional plakoglobin (JUP), Desmo

253 EKC syndrome subjects affect localization of desmosomal proteins and connexin 43 in the skin, and res

254 ia, with distinct effects on localization of desmosomal proteins and connexin 43.

255 urse comparable with the processing of other desmosomal proteins and cytoplasmic keratins.

256 biological functions that include degrading desmosomal proteins and inducing proinflammatory cytokin

257 ogenic right ventricular cardiomyopathy, and desmosomal proteins are targeted by pathogenic autoantib

258 Mutations in genes encoding for desmosomal proteins are the most common cause of arrhyth

259 keratin filaments, and increased turnover of desmosomal proteins at the cell cortex.

260 mine whether a change in the distribution of desmosomal proteins can be used as a sensitive and speci

261 emonstrated with pathogenic mutations in the desmosomal proteins desmoplakin and desmoglein 1.

262 s in DSP, JUP, PKP2, DSG2 and DSC2, encoding desmosomal proteins desmoplakin, plakoglobin, plakophili

263 PKP-1 prevents loss of Dsg3 and other desmosomal proteins from cell-cell borders and prevents

264 Desmosomal proteins from the ICD were decreased, consist

265 Mutations in genes encoding desmosomal proteins have been identified as the major ca

266 ngs highlight the importance of non-cadherin desmosomal proteins in modulating PV phenotypes and prov

267 However, the effects of non-cadherin desmosomal proteins in modulating the cellular manifesta

268 sis, we investigated the distribution of key desmosomal proteins in normal human and Darier's disease

269 merging evidence of supra-adhesive roles for desmosomal proteins in regulating tissue morphogenesis a

270 dition caused by mutations in genes encoding desmosomal proteins in up to 60% of cases.

271 osomes at 24 hours, with effects on multiple desmosomal proteins including Dsc3 and plakoglobin.

272 adherens junction protein p120ctn and to the desmosomal proteins plakophilins 1-3.

273 Other desmosomal proteins showed variable changes, but signal

274 In patient tissue, desmosomal proteins were aberrantly clustered and patien

275 ch is due to autoantibodies directed against desmosomal proteins) and in patients with Darier disease

276 heritable skin barrier defects, in this case desmosomal proteins, in the pathogenesis of atopic disea

277 resulted in a reduction in staining of other desmosomal proteins, including desmoglein 1 and 2, plako

278 an inherited disease involving mutations in desmosomal proteins, including PKP2.

279 ricular cardiomyopathy, a genetic disease of desmosomal proteins, is fibroadipocytic replacement of t

280 It mediates interactions with other desmosomal proteins, is found in a variety of plakin pro

281 ssion of N-cadherin, increased expression of desmosomal proteins, or a preferential expression of the

282 ent immunoreactive signals of PKP2 and other desmosomal proteins.

283 her expands the origin of the disease beyond desmosomal proteins.

284 disordered and forms an interaction hub for desmosomal proteins.

285 is a genetic disease caused by mutations in desmosomal proteins.

286 surface distribution of adherens junction or desmosomal proteins.

287 d immunostaining revealed an upregulation of desmosomal proteins.

288 senting intercalated discs with incorporated desmosomal proteins.

289 y minor changes in immunoreactivity of other desmosomal proteins.

290 l repercussions of debilitating mutations on desmosomal proteins.

291 ompromising barrier integrity, also leads to desmosomal remodeling and loss of the midline structure.

292 from barrier perturbation triggers transient desmosomal remodeling, seen as an increase in extracellu

293 ER Ca(2+) homeostasis also modulates normal desmosomal reorganization, both at rest and after acute

294 uggest that PV IgG binding to dsg3 activates desmosomal signal transduction cascades leading to (i) p

295 mphigus vulgaris IgG (PVIgG) to KCs induces "desmosomal" signaling.

296 ides a model system for molecular studies of desmosomal stability and keratinocyte adhesion, and for

297 d also suggest that JAM-C may play a role in desmosomal structure/function.

298 olynuclear areas that lacked cell membranes, desmosomal structures, and filamentous actin.

299 interactions and a proportion of rudimentary desmosomal structures.

300 ving probands, of whom 28% had an additional desmosomal variant (ie, mutation or polymorphism).