PLEC Antibody, FITC conjugated
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中文名稱：PLEC兔多克隆抗體, FITC偶聯
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貨號：CSB-PA619965LC01HU
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規格：￥880
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) PLEC Polyclonal antibody
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Uniprot No.：
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基因名：
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別名：EBS1 antibody; EBSO antibody; HD1 antibody; Hemidesmosomal protein 1 antibody; PCN antibody; pleC antibody; PLEC_HUMAN antibody; PLEC1 antibody; PLEC1b antibody; Plectin 1 antibody; Plectin 1 intermediate filament binding protein 500kDa antibody; Plectin 6 antibody; Plectin antibody; Plectin-1 antibody; PLTN antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Plectin protein (1744-1929AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：FITC
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 -
產品提供形式：Liquid
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
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靶點詳情
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功能：Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofiber integrity.
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基因功能參考文獻：
- The study has identified two mutations in two large consanguineous pedigrees. Identification of novel variants in the LAMA3 and PLEC genes will expand the mutation spectrum and also help in genetic counselling of patients in the Pakistani population. PMID: 29797489
- These findings extend current knowledge of the mutation spectrum of the PLEC gene associated with limbgirdle muscular dystrophy 2Q. PMID: 28447722
- The Structure of the Plakin Domain of Plectin Reveals an Extended Rod-like Shape. PMID: 27413182
- We present two cases of Epidermolysis bullosa with significant urologic involvement resulting from mutations in plectin. PMID: 27813154
- lectin and cytoskeletons were not detected in the nuclei of liver cells compared to the results of confocal microscopy. Despite the absence of nuclear plectin and cytoskeletal filaments, the evidence provided support that nuclear pleomorphism of cancer cells is correlated with the cytoplasmic disorganization of cytoskeleton. PMID: 27566071
- data demonstrate that plectin is an essential regulator of nuclear morphology in vitro and in vivo and protects the nucleus from mechanical deformation. PMID: 26527396
- The dominant expression of the P1a isoform in epidermal basal cell layer and cultured keratinocytes suggests that mutations in the first exon of isoform 1a cause skin-only epidermolysis bullosa simplex without extracutaneous involvement. PMID: 25712130
- Report of a non-consanguineous Iranian family with two affected sisters showing limb-girdle muscular dystrophy and myasthenic symptoms without any skin involvement, caused by plectinopathy. PMID: 25556389
- plectin interacts with keratins 5 and 14 in a process associated with epidermolysis bullosa simplex PMID: 24940650
- Six of sicteen epidermolysis bullosa simplex probands had dominant PLEC missense mutations. PMID: 23774525
- Mislocated Plectin is necessary for exosome formation and produces exosomes that are capable of promoting pancreatic tumor growth and aggression. PMID: 24218614
- this study is the first to demonstrate that up-regulation of vimentin and plectin expression positively correlates with the invasion and metastasis of androgen-independent PCA PMID: 23717685
- the potential importance of the endophilin B2-plectin complex in the biological functions depending on nuclear migration and positioning. PMID: 23921385
- results confirm epidermolysis bullosa simplex-pyloric atresia is linked to mutations in distal exons 1-30 and 32 of PLEC; while epidermolysis bullosa simplex-muscular dystrophy is linked to PLEC mutations in all exons, in most cases one of the mutations affects exon 31 PMID: 23289980
- findings suggest that plectin promotes the migration and invasion of head and neck squamous cell carcinoma (HNSCC) cells through activation of Erk 1/2 kinase and is a potential prognostic biomarker of HNSCC. PMID: 22245045
- mutational analysis of PLEC1 revealed a homozygous 36 nucleotide insertion (1506_1507ins36) that results in a reduced expression of PLEC1 mRNA and plectin in patients with epidermolysis bullosa simplex and congenital myasthenic syndrome. PMID: 21175599
- A linker region in the COOH-terminal end and serine residue at position 4645 may be important for the binding of plectin to intermediate filaments. PMID: 22333906
- The novel FUS-plectin interaction offers new perspectives for understanding the role of FUS and plectin mutations in the pathogenesis of FUS associated diseases PMID: 22240165
- novel esophageal squamous cell carcinoma marker plectin 1 was identified by mass spectrometry and immunohistochemical analysis PMID: 21743296
- plectin participates in actin assembly and invasiveness in carcinoma cells in an isoform-specific manner PMID: 21821021
- plectin deficiency might play an important role in the transformation of human liver cells PMID: 21424933
- The structure of the plakin domain of plectin reveals a non-canonical SH3 domain interacting with its fourth spectrin repeat. PMID: 21288893
- degradation of plectin induced by staurosporine-treatment in liver cells resulted in cytoskeleton disruption and induced morphological changes in these cells by affecting the expression and organization of cytokeratin 18. PMID: 21420381
- plectin expression was deficient in hepatocellular carcinoma and was probably through post-translational modification PMID: 21469503
- Plectin deficiency can indeed lead to both muscular dystrophy and pyloric atresia in an individual epidermolysis bullosa simplex (EBS) patient. PMID: 20665883
- data show that isoform 1f of plectin plays a key role in skeletal muscle function and that disruption of the plectin 1f can cause the LGMD2 phenotype without any dermatologic component as was previously reported with mutations in constant exons of PLEC PMID: 21109228
- Plectin expression patterns determine two distinct subtypes of epidermolysis bullosa simplex PMID: 20052759
- Autosomal recessive forms of epidermolysis bullosa simplex associated with extracutaneous manifestations, such as muscular dystrophy (MIM 226670) or pyloric atresia (MIM 612138), have been linked to genetic mutations in the gene for plectin PMID: 20447487
- missense mutation causes structure of plectin amino terminus alteration so binding to actin and costameric proteins are attenuated PMID: 20016501
- The structure was solved by the molecular-replacement method. In addition, the preparation of selenomethionine-derivative crystals is described. PMID: 12136158
- Data report the crystal structure of the actin binding domain of plectin and show that this region is sufficient for interaction with F-actin or the cytoplasmic region of integrin alpha6beta4. PMID: 12791251
- propose a previously unrecognized function of plectin as cytoskeletal regulator of PKC signaling (dislocation of PKCdelta and elevated enzymatic activity), and possibly other signaling events, through sequestration of the scaffolding protein RACK1 PMID: 14966116
- the involvement of desmoplakin (DP), plectin, and periplakin in the destruction of epithelial cell integrity ensures the efficient elimination of cytoskeleton, but also provides specificity for selectively targeting individual adhesion molecules PMID: 15500642
- homozygous mutations in the plectin gene (PLEC1)in epidermolysis bullosa with pyloric atresia PMID: 15654962
- discussion of phenotypic spectrum of plectin mutations [review] PMID: 15810881
- the CH1 domain of the plectin-ABD associates with the groove between the two FNIII domains of beta4 PMID: 15817481
- Co-localization, co-immunoprecipitation, and in vitro overlay analyses demonstrated direct interaction of plectin and GFAP. PMID: 16507904
- Results describe the crystal structure of an N-terminal fragment of the plakin domain of plectin to 2.05 A resolution. PMID: 17397861
- PLEC1 was differeentially expressed in sclerotic hippocampi compared to non-sclerotic ones. PMID: 17515952
- A novel functional co-localisation is identified between two plakin cytolinker proteins. PMID: 17662978
- results reveal that plectin is up-regulated in colorectal adenocarcinoma as well as in bizarre glands and locally invasive tumor nests in tubular adenoma, compared with normal colorectal mucosa PMID: 18084872
- Thus, plectin appears to interact with CXCR4 and plays an important role in CXCR4 signaling and trafficking and HIV-1 infection. PMID: 18155192
- Data show that intermediate filament recruitment to focal adhesions in endothelial cells requires beta3 integrin, plectin and the microtubule cytoskeleton, and is dependent on microtubule motors. PMID: 19366731
- BRCA2-plectin interaction plays an important role in the regulation of centrosome localization and also that displacement of the centrosome may result in genomic instability and cancer development. PMID: 19709076
- During polarization of peripheral blood T lymphocytes, plectin redistributes to the uropod associated with vimentin and fodrin. This vimentin-plectin-fodrin complex provides a continuous linkage from the nucleus (lamin B) to the cortical cytoskeleton. PMID: 11441066
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相關疾病：Epidermolysis bullosa simplex with pyloric atresia (EBS-PA); Epidermolysis bullosa simplex, with muscular dystrophy (MD-EBS); Epidermolysis bullosa simplex, Ogna type (O-EBS); Limb-girdle muscular dystrophy 2Q (LGMD2Q); Epidermolysis bullosa simplex with nail dystrophy (EBSND)
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亞細胞定位：Cytoplasm, cytoskeleton. Cell junction, hemidesmosome.
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蛋白家族：Plakin or cytolinker family
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組織特異性：Widely expressed with highest levels in muscle, heart, placenta and spinal cord.
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數據庫鏈接：
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