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

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

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

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

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

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

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

17 c autoantibodies mainly directed against two desmosomal adhesion proteins, desmoglein (Dsg)1 and Dsg3

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 igus vulgaris cause blisters through loss of desmosomal adhesion.

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

22 is required for establishing and maintaining desmosomal adhesion.

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

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

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

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

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

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

29 Desmosomal and LMNA gene variants identify the subset of

30 Carriers of desmosomal and LMNA variants experienced the highest rat

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

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

33 The resulting model of the desmosomal architecture explains their unique biophysica

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

56 in turn promotes differentiation through the desmosomal cadherin Dsg1.

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

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

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

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

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

62 Sporadic BCCs also overexpress Dsg2, a desmosomal cadherin normally found in the basal layer.

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

64 We previously showed that a desmosomal cadherin promotes keratinocyte differentiatio

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

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

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

68 Differential regulation of desmosomal cadherin transport could provide a mechanism

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

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

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

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

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

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

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

76 alloproteinase-dependent proteolysis of this desmosomal cadherin.

77 at is similar to that of adherens junctions, desmosomal cadherins - called desmogleins and desmocolli

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

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

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

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

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

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

84 The desmosomal cadherins are calcium-dependent transmembrane

85 Desmosomal cadherins are transmembrane adhesion molecule

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

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

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

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

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

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

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

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

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

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

96 Although the structure of the desmosomal cadherins is known, the desmosome architectur

97 an ex vivo human skin model, suggesting that desmosomal cadherins may have different roles during acq

98 Desmosomal cadherins mediate cell-cell adhesion in epith

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

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

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

102 mportant property, specific contributions of desmosomal cadherins to intestinal mucosal repair after

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

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

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

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

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

108 The desmosomal cadherins, comprising the desmogleins and des

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

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

111 ithelial cells (IEC) exclusively express the desmosomal cadherins, Desmoglein-2 and Desmocollin-2 (Ds

112 Desmosomal cadherins, desmogleins (Dsgs) and desmocollin

113 The desmosomal cadherins, desmogleins (Dsgs) and desmocollin

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

115 The desmosomal cadherins, desmogleins, and desmocollins medi

116 In contrast, antibodies against desmosomal cadherins, including human and mouse pemphigu

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

118 ), which is caused by autoantibodies against desmosomal cadherins, often have dry eye disease.

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

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

121 ness depends on the organised arrangement of desmosomal cadherins.

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

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

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

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

126 ensus sequence not conserved among the other desmosomal cadherins.

127 In turn, desmosomal catenins interact with members of the IF-bind

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

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

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

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

132 and the stratum corneum was detached through desmosomal cleavage.

133 ) serves to anchor intermediate filaments in desmosomal complexes.

134 teolysis of corneodesmosin, an extracellular desmosomal component.

135 Localization of other desmosomal components appears normal, which is in contra

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

137 In this Minireview, we discuss when and how desmosomal components evolved, and how their ability to

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

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

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

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

142 Desmosomal components were downregulated, consistent wit

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

167 Multiple desmosomal gene mutations and male sex were independent

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

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

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

171 Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were ide

172 e genotyping that included sequencing of the desmosomal genes (PKP2, DSP, DSG2, DSC2, and JUP) from 3

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

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

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

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

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

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

179 Screening of 5 desmosomal genes was performed in probands; when a seque

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

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

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

183 tricular ACM with more a diverse etiology in desmosomal genes.

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

185 uctural cytoskeleton-Z disk genes; 16 (3.5%) desmosomal genes; 46 (9.5%) sarcomeric genes; 8 (1.6%) i

186 rtance of heart examination of patients with desmosomal genodermatoses.

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

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

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

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

191 ithelial barrier (IEB) function with loss of desmosomal junctional protein desmoglein 2 (DSG2) is a h

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

193 Desmosomal localization of JAM-C was further confirmed b

194 atinocyte cell surfaces and colocalized with desmosomal markers.

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

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

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

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

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

200 +/- 17 years; 18 males) family members of 12 desmosomal mutation-carrying ARVD/C probands underwent g

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

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

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

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

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

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

207 compared with other genes (PKP2, n=1, 8.3%; desmosomal non-PKP2, n=20, 66.7%; nondesmosomal, n=26, 6

208 ; P=0.001for overall comparison; PKP2 versus desmosomal non-PKP2, P=0.001; PKP2 versus nondesmosomal,

209 Most desmosomal P/LP variants are inherited, nonunique, and o

210 iomyopathy probands, 322 (64.3%) carried 327 desmosomal P/LP variants.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

231 primary heart muscle disorder resulting from desmosomal protein mutations.

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

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

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

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

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

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

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

239 the heart) and PKP2 (the gene coding for the desmosomal protein plakophilin-2), as well, is discussed

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

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

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

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

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

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

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

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

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

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

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

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

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

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

254 We show that the desmosomal proteins desmoglein-2 and desmocollin-3, the

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

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

257 Desmosomal proteins from the ICD were decreased, consist

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

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

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

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

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

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

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

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

266 Other desmosomal proteins showed variable changes, but signal

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

268 ss spectrometry analysis identified all core desmosomal proteins while uncovering a diverse array of

269 heart disease linked to mutations in several desmosomal proteins, but the specific effects of these m

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

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

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

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

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

275 Knockdown of two desmosomal proteins, junction plakoglobin or desmocolin-

276 Finally, we observed decreases in desmosomal proteins, plakophilin-2 and desmoglein-2, whi

277 rphine increased the proximity of the MOR to desmosomal proteins, which form specialized and highly-o

278 y minor changes in immunoreactivity of other desmosomal proteins.

279 l repercussions of debilitating mutations on desmosomal proteins.

280 ent immunoreactive signals of PKP2 and other desmosomal proteins.

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

282 disordered and forms an interaction hub for desmosomal proteins.

283 P/LP) variants in genes encoding the cardiac desmosomal proteins.

284 itions caused by mutations in genes encoding 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 The expression profiles of the desmosomal PV antigens desmoglein (Dsg) 3 and 1 but not

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

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

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

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

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

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

296 Desmosomal structure and function appeared preserved in

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

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

299 ts that cause dilated cardiomyopathy and the desmosomal variants that cause either arrhythmogenic rig

300 We classified the desmosomal variants, defined the contribution of unique