ライフサイエンスコーパス: desmoplakin

1 gesting that p0071 may couple VE-cadherin to desmoplakin.

2 he intermediate filament-associated protein, desmoplakin.

3 le colocalizes with beta-catenin rather than desmoplakin.

4 examined by immunofluorescence for pinin and desmoplakin.

5 port the first mutation in the gene encoding desmoplakin.

6 skeleton, presumably through plakoglobin and desmoplakin.

7 in response to RSV, including annexin A2 and desmoplakin.

8 internalization of Dsg3, desmocollin 3, and desmoplakin.

9 ediate filament cytoskeleton, linked through desmoplakin.

10 diate filaments through its association with desmoplakin.

11 urface in a complex with plakoglobin but not desmoplakin.

12 d formed complexes with both VE-cadherin and desmoplakin.

13 Desmoplakin, a constitutive component of the desmosomal

14 amounts of involucrin epitopes as well as of desmoplakin, a desmosomal structural protein.

15 DSP encodes desmoplakin, a primary component of desmosomes, intercel

16 he microtubule network and the N terminus of desmoplakin, a region that is a pathogenic mutation hots

17 ssays, plakophilin-1 formed complexes with a desmoplakin amino-terminal domain and enhanced its recru

18 to the first 160 amino-terminal residues of desmoplakin and also interacted directly with plakoglobi

19 unstable proteins that fail to interact with desmoplakin and are targeted by degradation involving ca

20 es, we have assigned particular densities to desmoplakin and described their interaction with interme

21 iASPP interacts with desmoplakin and desmin in cardiomyocytes to maintain the

22 se data demonstrate that by interacting with desmoplakin and desmin, iASPP is an important regulator

23 oglobin and plakophilin and more weakly with desmoplakin and desmocollin 1.

24 hogenic mutations in the desmosomal proteins desmoplakin and desmoglein 1.

25 of epithelial specific genes E-cadherin and desmoplakin and induces the expression of the mesenchyma

26 sulting from a decrease in binding sites for desmoplakin and intermediate filaments at desmosomes.

27 esion because mutations in desmosomal genes, desmoplakin and plakoglobin, have been implicated in the

28 globin interactions, the interaction between desmoplakin and plakophilin-1 was not mediated by the ar

29 ized periplakin, and certain sera recognized desmoplakin and plectin, and, weakly, bullous pemphigoid

30 immunoprecipitation approaches we found that desmoplakin and talin2 mRNAs associate with FXR1 in a co

31 Fxr1 KO hearts exhibit an up-regulation of desmoplakin and talin2 proteins, which is accompanied by

32 requirements for direct interactions between desmoplakin and various IF types.

33 Homozygous DSP (desmoplakin) and JUP (junction protein plakoglobin) muta

34 kedly decreased localization of plakoglobin, desmoplakin, and connexin43 at intercalated discs in car

35 highlighted by the expression of E-cadherin, desmoplakin, and cytokeratins.

36 at desmosomes and had become cross-linked to desmoplakin, and in 5-d CEs, these three proteins had fo

37 he mechanical junction proteins plakoglobin, desmoplakin, and N-cadherin are also upregulated by puls

38 ce with antibodies to the desmosome protein, desmoplakin, and the adherens junction protein, vinculin

39 interactions among the desmosomal cadherins, desmoplakin, and the armadillo family members plakoglobi

40 G15 [interferon-stimulated gene 15 kd], DSP [Desmoplakin], and C1S [complement component 1s subcomple

41 It identifies dosage of desmoplakin as critical in maintaining epidermal integri

42 Kazrin colocalizes with periplakin and desmoplakin at desmosomes and with periplakin at the int

43 the intermediate filament-associated protein desmoplakin at the carboxyl terminus.

44 presence of markers for cell junction (ZO1, Desmoplakin), basement membrane assembly (Collagen 7, La

45 kin family of proteins, to which envoplakin, desmoplakin, bullous pemphigoid antigen 1, and plectin b

46 m a PV patient causes a decrease of Dsg3 and desmoplakin but not desmocollin (Dsc) 3 in the Triton-in

47 finding not only unveiled a new function for desmoplakin, but also provided the first opportunity to

48 Mutations in desmoplakin can result in devastating skin blistering di

49 ve also characterized fully the 3'UTR of the desmoplakin cDNA.

50 Immunofluorescence for desmoplakin confirmed desmosome presence throughout all

51 ession of other ID proteins like N-cadherin, desmoplakin, connexin-43, and ZO-1 was significantly per

52 discs, with disturbed expression of desmin, desmoplakin, connexin43 and vinculin being evident.

53 Mutation screening of desmoplakin demonstrated compound heterozygosity for a n

54 cell adhesion via impaired redistribution of desmoplakin, desmoglein 3, desmocollin 3, and E-cadherin

55 keratinocytes impairs the trafficking of the desmoplakins, desmoglein, and desmocollin to the cell su

56 the ability to mediate the initial phase of desmoplakin (DP) accumulation at sites of cell-cell cont

57 Desmoplakin (DP) anchors the intermediate filament cytos

58 me precursors enriched in the plaque protein desmoplakin (DP) into newly forming desmosomes, in part

59 Desmoplakin (DP) is an integral part of desmosomes, wher

60 mplicated in making this IF connection, only desmoplakin (DP) is both exclusive to and ubiquitous amo

61 e intermediate filament (IF)-binding protein desmoplakin (DP) is essential for desmosome function and

62 portantly, we demonstrate that expression of Desmoplakin (DP) is lost upon PKP2 knockdown and that re

63 paper, we report that the desmosomal protein desmoplakin (DP) is not essential for cell adhesion in t

64 ted Tet-On A431 cells inducibly expressing a desmoplakin (DP) mutant lacking the rod and IF-binding d

65 termediate filaments, the desmosomal protein desmoplakin (DP) regulates microtubule reorganization in

66 Desmoplakin (DP) serves to anchor intermediate filaments

67 al modifications on the IF-anchoring protein desmoplakin (DP) that play an essential role in coordina

68 s, which interface with plakoglobin (PG) and desmoplakin (DP) to associate with the intermediate fila

69 e intermediate filament (IF)-binding protein desmoplakin (DP) to desmosomal cadherins.

70 te differentiation, E-cadherin localization, desmoplakin (DP) translocation, and ER Ca(2+) sequestrat

71 d-binding 1 (EB1) and the desmosomal protein desmoplakin (DP), and demonstrate that DP-EB1 interactio

72 H terminus of the desmosomal plaque protein, desmoplakin (DP), is required for the association of DP

73 The desmosomal plaque protein desmoplakin (DP), located at the juncture between the in

74 Desmoplakin (DP), plakoglobin (PG), and plakophilin 1 (P

75 myocyte cell lines expressing siRNA against desmoplakin (DP), responsible for human ARVC.

76 o a 60% reduction in border translocation of desmoplakin (DP), the desmosomal cytolinker protein nece

77 esmosomal components, Desmoglein1 (Dsg1) and Desmoplakin (Dp), to promote epidermal differentiation.

78 er essential intercellular adhesion protein, desmoplakin (DP).

79 centrosome and is recruited to desmosomes by desmoplakin (DP).

80 subjects with PKP2 variants (42%), including desmoplakin (DSP) (n = 6), desmoglein-2 (DSG2) (n = 5),

81 omal proteins (Junctional plakoglobin (JUP), Desmoplakin (DSP), Plakophilin 2, and Desmoglein 2), hav

82 , OR 2.89 [2.56-3.26], p=1.12 x 10(-66)) and desmoplakin (DSP; rs2076295, OR 1.44 [1.35-1.54], p=7.81

83 emonstrate an interaction between SERCA2 and desmoplakin during differentiation.

84 rotein components, including cystatin alpha, desmoplakin, elafin, keratins, members of the small prol

85 oricrin, and unknown proteins related to the desmoplakin family.

86 proteins include alpha tubulin, beta actin, desmoplakin, fibrillarin, nuclear lamin B1, nonmuscle my

87 is significantly inhibited; in these cells, desmoplakin forms insoluble aggregates when extracted wi

88 on of PG, which was accompanied by a loss of desmoplakin from desmosomes and decreased adhesive stren

89 prevented the EGF receptor-dependent loss of desmoplakin from junctions, attenuating the effects of l

90 ion at the donor + 1 site of intron 7 of the desmoplakin gene (939 + 1 G > A; Genbank M77830).

91 erozygous missense c.1757A>C mutation in the desmoplakin gene (DSP) was identified in the patient, pr

92 terozygous C-->T transition in exon 4 of the desmoplakin gene and predicted a premature termination c

93 reaction the exon-intron organization of the desmoplakin gene to facilitate analysis of genomic DNA.

94 t recessive human mutation, 7901delG, in the desmoplakin gene which causes a generalized striate kera

95 portance to tissue integrity, we ablated the desmoplakin gene.

96 uctures of the plakin domains of plectin and desmoplakin give insight into how different plakins migh

97 proteins, such as glutamines 495 and 496 to desmoplakin, glutamine 288 to keratins, and lysines 468,

98 SI region that is involved in the binding to desmoplakin, had no effect.

99 tational analyses therefore demonstrate that desmoplakin haploinsufficiency can be tolerated in some

100 In contrast, desmoplakin has a two-segment structure with a central f

101 The flexibility within desmoplakin has been revealed by small-angle X-ray scatt

102 gene encoding the desmosomal plaque protein, desmoplakin, has been described in a patient with autoso

103 Recently, mutations in desmoplakin have been shown to underlie some cases of th

104 in(s) that bind the amino-terminal domain of desmoplakin have not been identified.

105 he intermediate filament-associated proteins desmoplakin I (DPI) and plectin.

106 re identified (desmoglein 3, desmocolin A/B, desmoplakin I, plakoglobin, and plakophilin), indicating

107 andomly distributed around the cell surface, desmoplakins I/II accumulate intracellularly, and the ti

108 In addition, while both ZO-1 and desmoplakin-I in control cells were shown to become asso

109 ters Dsg3 with the desmosomal plaque protein desmoplakin in a manner dependent on the plakoglobin-bin

110 oncomitantly deleting the desmosomal protein desmoplakin in cardiac myocyte lineages using the Cre-Lo

111 basis for understanding the critical role of desmoplakin in desmosome function.

112 These results identify a role for desmoplakin in organizing the desmosomal cadherin-plakog

113 This study demonstrates the importance of desmoplakin in the attachment of intermediate filaments

114 ates the relevance of haploinsufficiency for desmoplakin in the pathogenesis of this genodermatosis.

115 on was paralleled by an increase in Dsg2 and desmoplakin in the Triton-insoluble cell fraction, sugge

116 evealed that VE-cadherin colocalization with desmoplakin in transfected COS cells required p0071, sug

117 scopy and immunofluorescence localization of desmoplakin, into punctate structures.

118 Desmoplakin is a cytoplasmic desmosomal protein that pla

119 Desmoplakin is a desmosomal component that plays a criti

120 This suggests that the tail domain of desmoplakin is not required for establishing tissue arch

121 sease keratinocytes, only the trafficking of desmoplakin is significantly inhibited; in these cells,

122 diac-specific loss of the desmosomal protein desmoplakin is sufficient to cause nuclear translocation

123 osome consists of several proteins, of which desmoplakin is the most abundant.

124 the expression of a cell-cell contact gene, Desmoplakin, is greatly reduced in Norbin-deletion mice.

125 ize and contain a dramatic redistribution of desmoplakin, keratin 5, and chromogranin A proteins.

126 d of cells with elongated morphology and low desmoplakin levels, suggesting a role in morphogenetic c

127 velopment or homeostasis, and periplakin and desmoplakin localization was normal.

128 epidermal differentiation complex on 1q, the desmoplakin locus on 6p, the type I and II keratin gene

129 In contrast to null DESMOPLAKIN: mice which die in early development, the tr

130 Desmoplakin molecules are more extended in wild type tha

131 ilin-1 enhances lateral interactions between desmoplakin molecules.

132 ARVC-derived desmoplakin mutants DSP-1-V30M and DSP-1-S299R exhibit w

133 We now report a further case of a desmoplakin mutation in a proband with striate palmoplan

134 Plakophilin and desmoplakin mutations have been discovered to alter bind

135 The desmoplakin N-terminal region is a 1056-amino-acid seque

136 carried a single desmosomal gene mutation in desmoplakin (n=44; 39%), plakophilin-2 (n=38; 34%), desm

137 f these embryos revealed a critical role for desmoplakin not only in anchoring IFs to desmosomes, but

138 l keratins show elevated, PKC-alpha-mediated desmoplakin phosphorylation and subsequent destabilizati

139 rP(c) knockdown cells; levels of E-cadherin, desmoplakin, plakoglobin, claudin-4, occludin, zonula oc

140 ith several desmosomal components, including desmoplakin, plakoglobin, desmoglein 1 and 2, and desmoc

141 DSG2 and DSC2, encoding desmosomal proteins desmoplakin, plakoglobin, plakophilin 2 (PKP2), desmogle

142 ts of desmosomes (plakophilin (PKP) 1 and 2, desmoplakin, plakoglobin--which is also present in adher

143 In contrast to desmoplakin-plakoglobin interactions, the interaction be

144 tions with plakoglobin, the plakin domain of desmoplakin, plakophilin 1, and the cytoplasmic domain o

145 previous findings that both VE-cadherin and desmoplakin play central roles in vasculogenesis, these

146 The constitutive desmosomal plaque protein desmoplakin plays a vital part in keratinocyte adhesion

147 owever, in the presence of an amino-terminal desmoplakin polypeptide (DP-NTP), the desmosomal cadheri

148 e amino acid substitution p.His586Pro in the desmoplakin polypeptide.

149 FAPs expressed desmosome proteins, including desmoplakin, predominantly in the adipogenic but not fib

150 premature stop codon leading to a truncated desmoplakin protein missing the C domain of the tail reg

151 change in the distribution of pinin, whereas desmoplakin remained cell boundary-associated.

152 Herein we elucidate the architecture of desmoplakin's plakin domain, as well as its constituent

153 ECM remodeling genes include desmoplakin, SPARCL1, biglycan, and PECAM.

154 mal plaque, and the amino-terminal domain of desmoplakin targets desmoplakin to the desmosome.

155 to the plakin family of proteins and include desmoplakin, the 230 kDa bullous pemphigoid antigen, and

156 n between DSMs and IFs using mutant forms of desmoplakin, the protein bridging these structures.

157 n interact with the amino-terminal domain of desmoplakin, these proteins were co-expressed in L-cell

158 leus and enhancing the recruitment of excess desmoplakin to cell borders in transiently transfected C

159 osomal plaque components, in which they link desmoplakin to the desmosomal cadherins.

160 amino-terminal domain of desmoplakin targets desmoplakin to the desmosome.

161 were necessary for subsequent recruitment of desmoplakin to the membrane and desmosome assembly.

162 , followed by recruitment of plakophilin and desmoplakin to the plaque, and ending with anchoring of

163 o recruit normal levels of plakophilin-1 and desmoplakin to the plaque.

164 In contrast to VE-cadherin, desmoplakin was found to associate with the non-armadill

165 from both affected individuals revealed that desmoplakin was not just located at the cell periphery b

166 Signal for the desmosomal protein desmoplakin was reduced in buccal mucosa cells from pati

167 delay in sorting of the desmosomal protein, desmoplakin, was observed in the presence of TG.

168 serves as a linker between the cadherins and desmoplakin, whereas plakophilin-1 enhances lateral inte