ライフサイエンスコーパス: plectin

1 oskeleton to intermediate filaments (such as plectin).

2 antibodies as well as by antibodies against plectin.

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

4 res of vimentin IFs with sidearms containing plectin.

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

6 , a protein known to interact with cytosolic plectin.

7 o intermediate filaments by interacting with plectin.

8 ates the formation of a binding platform for plectin.

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

10 ignificantly attenuated in AECs deficient in plectin.

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

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

13 Plectin 1 or endophilin B2 knockdown using RNA interfere

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

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

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

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

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

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

20 1.2%; OR: 1.55; p = 8.0 x 10(-10)), encoding plectin, a cytoskeletal cross-linking protein that contr

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

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

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

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

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

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

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

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

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

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

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

32 analyses demonstrated direct interaction of plectin and GFAP.

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

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

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

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

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

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

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

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

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

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

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

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

45 Plectin associates with a complex of structural proteins

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

47 Plectin binding to microtubules was significantly increa

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

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

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

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

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

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

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

55 a homozygous frameshift mutation detected in plectin cDNA.

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

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

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

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

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

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

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

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

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

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

66 Because plectin deficiency is associated with muscular dystrophy

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

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

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

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

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

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

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

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

75 Plectin-deficient myotubes, derived from myoblasts, were

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

77 Moreover, plectin expression was necessary for efficient exosome p

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

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

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

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

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

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

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

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

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

87 ive disorder resulting from mutations in the plectin gene.

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

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

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

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

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

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

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

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

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

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

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

99 Plectin is a prototypical plakin that tethers intermedia

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

101 Plectin is a widely expressed high molecular weight prot

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

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

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

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

106 Plectin is therefore likely to serve as an important org

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

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

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

110 Plectin knockdown reduces DG interaction with AMPK but n

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

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

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

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

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

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

117 Plectin localization and hemidesmosome organization are

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

119 Rodless plectin mice develop normally without signs of skin blis

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

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

122 osome organization are unaffected in rodless plectin mice.

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

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

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

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

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

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

129 consanguineous MD-EBS family with intragenic plectin polymorphisms.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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