ライフサイエンスコーパス: plectin

1 oskeleton to intermediate filaments (such as plectin).

2 myosin-9, fructose-bisphosphate aldolase and plectin).

3 antibodies as well as by antibodies against plectin.

4 the end of N-terminal homology with DPI and plectin.

5 res of vimentin IFs with sidearms containing plectin.

6 -associated proteins desmoplakin I (DPI) and plectin.

7 pathogenic variants in PLEC1, which encodes plectin.

8 , a protein known to interact with cytosolic plectin.

9 o intermediate filaments by interacting with plectin.

10 ates the formation of a binding platform for plectin.

11 in, which lacks binding sites for BPAG1e and plectin.

12 ignificantly attenuated in AECs deficient in plectin.

13 r, these data suggest that the endophilin B2-plectin 1 complex functions as a membrane-anchoring devi

14 nce for the involvement of endophilin B2 and plectin 1 in nuclear positioning in individual cells.

15 Plectin 1 or endophilin B2 knockdown using RNA interfere

16 n B2 directly binds the N-terminal region of plectin 1 via Src homology 3-mediated interaction and vi

17 nravel the interaction of endophilin B2 with plectin 1, a variant of the cytoskeleton linker protein

18 om both human and porcine TM cells contained Plectin 1, Filamin A, non-muscle myosin IIA, clathrin, a

19 Plectin-1 (Plec-1) was recently identified as specific b

20 We show here that plectin 1c (P1c), one of the multiple isoforms of the IF

21 The expression of plectin, a cytolinker protein that plays an important ro

22 1.2%; OR: 1.55; p = 8.0 x 10(-10)), encoding plectin, a cytoskeletal cross-linking protein that contr

23 Depletion of plectin, a cytoskeletal linker, partially prevented the

24 We report that mutation in the gene for plectin, a cytoskeleton-membrane anchorage protein, is a

25 hemidesmosomal inner plaque, which contains plectin, a high molecular weight cytoskeletal associated

26 ive for staining with antibodies recognizing plectin, a large cytoskeleton-associated anchorage prote

27 Here, we show that plectin, a major cytoskeletal crosslinker protein, plays

28 Here, we show that plectin, a major intermediate filament-based cytolinker

29 One of the 'hit' peptoids bound to plectin, a structural protein, predominantly expressed i

30 al receptor and scaffold for ERK1/2, whereas plectin acts as a scaffold for AMPK signaling but is als

31 orms of the IF-associated cytolinker protein plectin, acts as an MT destabilizer.

32 human peripheral blood T lymphocytes express plectin, an IF-binding, cytoskeletal cross-linking prote

33 ilar to the COOH terminus of plakins such as plectin and BPAG1e.

34 egmented structures of the plakin domains of plectin and desmoplakin give insight into how different

35 We demonstrate that plectin and DG form a complex in AECs and that this comp

36 apo, a large cytoskeletal protein related to plectin and dystrophin; flamingo, a seven-transmembrane

37 RFs displayed positive immunostaining for plectin and GFAP, both of which were increased in the Ax

38 analyses demonstrated direct interaction of plectin and GFAP.

39 enotypic and phenotypic correlations between plectin and lung CSCs, as well as association of high pl

40 The plakin domains of plectin and other plakins harbor binding sites for junct

41 n-3 is required for the localization of both plectin and vimentin at the nuclear perimeter.

42 xtracellular matrix and the Z-discs (through plectin) and may play an important role in maintaining m

43 components include the alpha6beta4 integrin, plectin, and BPAGs (bullous pemphigoid antigens).

44 and certain sera recognized desmoplakin and plectin, and, weakly, bullous pemphigoid antigen 1.

45 in with the preceding SR is also observed in plectin, another plakin protein, but not in alpha-spectr

46 by negative immunofluorescence with an anti-plectin antibody (HD-1), associated with fragility of ba

47 omal degradation, an increase in sarcolemmal plectin appeared to confer protection on Dag1(Y890F/Y890

48 Here, we demonstrate that high levels of plectin are associated with localized and metastatic hum

49 Nesprin-3 and plectin are coexpressed in a variety of tissues, includi

50 lung adenocarcinoma, potentially identifying plectin as a biomarker for lung CSCs.

51 The function of plectin as a putative attachment protein also in the mus

52 fragility of basal keratinocytes, implicates plectin as critical for binding of intermediate keratin

53 ament vimentin and the actin-vimentin linker plectin as unexpected candidates.

54 a variant of the cytoskeleton linker protein plectin as well as with vimentin.

55 Plectin associates with a complex of structural proteins

56 smoplakin, bullous pemphigoid antigen 1, and plectin belong.

57 Plectin binding to microtubules was significantly increa

58 HDs near the cell margins, colocalizing with plectin but always excluding BPAGs, suggesting that phos

59 The evidence comes from absence of plectin by antibody staining in affected individuals fro

60 Our data demonstrate that rodless plectin can functionally compensate for the loss of full

61 e large multifunctional cytoskeleton protein plectin can simultaneously account for structural failur

62 Transient transfection of full-length plectin cDNA converted these aggregates to thin filament

63 Direct sequencing of PCR-amplified plectin cDNA from the patient's keratinocytes revealed a

64 The 14800-bp human plectin cDNA was cloned and sequenced.

65 a homozygous frameshift mutation detected in plectin cDNA.

66 Interestingly, we found that plectin cell surface localization was attributable to it

67 sition of hemidesmosomal components, such as plectin, collagen type XVII/BP180, and integrin alpha6 a

68 ransmembrane BPAG1e-binding protein, but not plectin, colocalize along the substratum-attached surfac

69 nents (Lamin B1, Lamin A/C, Sun1, Nesprin-3, Plectin) compared with controls.

70 the popular pLink algorithms on a calmodulin-plectin complex data set, as well as three additional, p

71 he potential importance of the endophilin B2-plectin complex in the biological functions depending on

72 We conclude that the DG-plectin complex plays a central role in transmitting mec

73 Here we investigated whether plectin contributes to the structural integrity of NMJs

74 In conclusion, our study demonstrates that plectin-controlled cytoarchitecture is a key determinant

75 structural evidence for the hypothesis that plectin cross-links elements of the cytoskeleton thus le

76 recessive epidermolysis bullosa simplex with plectin defects (n = 3) or with autosomal recessive dyst

77 Because plectin deficiency is associated with muscular dystrophy

78 Using this cell model, we demonstrated that plectin deficiency leads to increased intermediate filam

79 t only for alpha6beta4 integrin but also for plectin deficiency.

80 specific manner; forced expression of P1f in plectin-deficient cells rescued both compromised AChR cl

81 found in the Triton X-insoluble fraction of plectin-deficient fibroblasts than in wild-type fibrobla

82 gates in human primary astrocytes and murine plectin-deficient fibroblasts.

83 in plectin-deficient myotubes as well as in plectin-deficient mice.

84 bes differentiated ex vivo from immortalized plectin-deficient myoblasts revealed them to be highly m

85 elioration of the pathological phenotypes in plectin-deficient myotubes as well as in plectin-deficie

86 Plectin-deficient myotubes, derived from myoblasts, were

87 , associated with general destabilization of plectin-deficient sheets upon mechanical stress.

88 teomic and phosphoproteomic profiling linked plectin-dependent disruption of cytoskeletal networks to

89 ntin is recruited to the mitotic cortex in a plectin-dependent manner.

90 results suggest that insufficient amounts of plectin, due to RC GFAP expression, promote GFAP aggrega

91 LEC1 premature termination codons and induce plectin expression in EBS-MD primary keratinocytes and s

92 Gentamicin treatment was followed by plectin expression in the skin for at least 5 months.

93 Moreover, plectin expression was necessary for efficient exosome p

94 cidate the structure of the plakin domain of plectin, extending our previous analysis of the SR1 to S

95 e in the N-terminal regions of the BPAG1/DPI/plectin family.

96 al distribution indicates that this vimentin-plectin-fodrin complex provides a continuous linkage fro

97 uencing revealed homozygous mutations in the plectin gene (PLEC1), encoding another hemidesmosomal pr

98 osomal variant of EB due to mutations in the plectin gene (PLEC1).

99 ular dystrophy, molecular diagnostics of the plectin gene provides prognostic value in evaluation of

100 genetic analysis (localization of the human plectin gene to chromosome 8q24.13-qter and evidence for

101 h with a homozygous deletion mutation in the plectin gene, PLEC1.

102 ciated with mutations in both alleles of the plectin gene.

103 test (PTT), and/or direct sequencing of the plectin gene.

104 ive disorder resulting from mutations in the plectin gene.

105 By contrast, depletion of the cytolinker plectin had the opposite effect and promoted increased m

106 ns are rod-like segments and that SR3-SR9 of plectin has an extended shape with a small central kink.

107 nd S1364, which reduces the interaction with plectin; however, this event is insufficient to drive co

108 Overexpression of the plectin IF-binding domain in the T cell line Jurkat indu

109 emonstrate the important structural role for plectin in cytoskeleton-membrane adherence in both skin

110 nally compensate for the loss of full-length plectin in mice.

111 To gain insights into the role of plectin in prostate cancer growth and metastasis, we per

112 muscular dystrophy (EBS-MD) and mice lacking plectin in skeletal muscle display pathological desmin-p

113 xpression of the cytoskeletal linker protein plectin in the AxD brain.

114 The absence of plectin in the hemidesmosomes, as reflected by negative

115 ne-based and murine HCC models, we show that plectin inhibitor plecstatin-1 (PST) is well-tolerated a

116 Knock-down of plectin inhibits prostate cancer cell growth and colony

117 nce and immunoprecipitation, suggesting that plectin is a candidate gene/protein system for MD-EBS mu

118 s because, like full-length plectin, rodless plectin is a dimeric protein.

119 Plectin is a prototypical plakin that tethers intermedia

120 We recently demonstrated that plectin is a robust biomarker for pancreatic ductal aden

121 Plectin is a widely expressed high molecular weight prot

122 Collectively these findings demonstrate that plectin is an important regulator of prostate cancer cel

123 In normal physiology, plectin is an intracellular scaffolding protein, but we

124 rates that linkage to desmin IF networks via plectin is crucial for formation and maintenance of AChR

125 expressed multifunctional cytolinker protein plectin is essential for muscle fiber integrity and myof

126 f plectin using RNA interference showed that plectin is essential for the B Tat-induced translocation

127 strate that in an F-actin-dependent context, plectin is essential for the formation of the circumfere

128 Plectin is therefore likely to serve as an important org

129 We found plectin isoform 1f (P1f) to bridge AChRs and IFs via dir

130 The faster migration of rodless plectin keratinocytes is not due to altered biochemical

131 tin to the integrin beta4 subunit in rodless plectin keratinocytes.

132 Consistently, plectin knock-down cells have impaired metastatic coloni

133 Plectin knock-down further impairs aggressive and invasi

134 Plectin knock-down inhibited number of metastases per or

135 formed proteomic analysis of prostate cancer plectin knock-down xenograft tissues.

136 Plectin knockdown reduces DG interaction with AMPK but n

137 In conditional plectin knockout mice with gene disruption in muscle pre

138 ypothesis that the cytoskeleton cross-linker plectin, known to bind both DG and AMPK in muscle cells,

139 Mutations in the cytolinker protein plectin lead to grossly distorted morphology of neuromus

140 GFAP expression, RC GFAP expression lowered plectin levels in astrocytoma-derived stable transfectan

141 Our expression analyses revealed elevated plectin levels in liver tumors, which correlated with po

142 rotein, or in syncoilin, which together with plectin links desmin to the Z-disk.

143 id it depend upon the presence of MAP4 since plectin links were retained after specific immunodepleti

144 Plectin localization and hemidesmosome organization are

145 In the absence of plectin-mediated cytoskeletal cross-linking, the aberran

146 ated interaction and vimentin indirectly via plectin-mediated interaction.

147 Rodless plectin mice develop normally without signs of skin blis

148 healing occurred slightly faster in rodless plectin mice than in wild-type mice, and keratinocytes m

149 tion of the rod domain, we generated rodless plectin mice through conditional deletion of exon 31.

150 osome organization are unaffected in rodless plectin mice.

151 nd lung CSCs, as well as association of high plectin mRNA expression with poor patient survival in lu

152 In this study we report novel plectin mutations in two families with EB.

153 pidermolysis bullosa simplex associated with plectin mutations the values were 31.9% +/- 8.9 (p < 0.0

154 number of cases diagnosed as EBS are due to plectin mutations, and many cases result from de novo mu

155 by the abnormal organization of desmoplakin, plectin, N-cadherin, and connexin-43.

156 ncluding UDP glucuronosyltransferase (UGT1), plectin, neuronal nitric oxide synthase (NOS1), and gluc

157 ve imaging of MTs in P1c(-/-), as well as in plectin-null, cells revealed decreased MT dynamics.

158 s residue regulates the interaction with the plectin plakin domain.

159 ciated proteins, including keratin 5 (KRT5), plectin (PLEC), integrin alpha 6 (ITGalpha6) and integri

160 consanguineous MD-EBS family with intragenic plectin polymorphisms.

161 astrocytoma-derived stable transfectants and plectin-positive fibroblasts.

162 genetic and pharmacological inactivation of plectin potently suppressed the initiation and growth of

163 Thus the low expression level of plectin rather than the absence of the rod domain dictat

164 During chemokine-induced polarization, plectin redistributes to the uropod associated with vime

165 therapy or treatment is available to improve plectin-related or other forms of MFMs; therefore, we as

166 hemical properties because, like full-length plectin, rodless plectin is a dimeric protein.

167 a role in PDAC, and further understanding of plectin's contribution to PDAC could enable improved the

168 ns through an inhibitory function exerted by plectin's SH3 domain.

169 These plectin sidearms connect IFs to microtubules, the actin-

170 leave the production of a low-level rodless plectin splice variant unaffected.

171 Moreover, plectin targeting potently inhibited the invasion potent

172 sely associated with them may play a role as plectin targets.

173 for BPAG1-e, as well as reduced labeling for plectin, the beta4 integrin subunit, and for type XVII c

174 a closer juxtaposition of the C-terminus of plectin to the integrin beta4 subunit in rodless plectin

175 esprin-3 is critical for the localization of plectin to the nuclear perimeter of Sertoli cells, the r

176 ytic inclusions that contain GFAP, vimentin, plectin, ubiquitin, Hsp27 and alphaB-crystallin.

177 Knockdown of plectin using RNA interference showed that plectin is es

178 Of interest, plectin was concentrated at the nuclear envelope in only

179 Here, plectin was found to be absent in skin and cultured kera

180 nesprin-3 in the perinuclear localization of plectin, we generated nesprin-3-knockout mice and examin

181 e results suggest the existence of a pool of plectin which preferentially associates with IFs but may