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anti-Human Plakophilin 2 Anticorps:
anti-Mouse (Murine) Plakophilin 2 Anticorps:
anti-Rat (Rattus) Plakophilin 2 Anticorps:
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Human Polyclonal Plakophilin 2 Primary Antibody pour IF (p), IHC (p) - ABIN1386389
Akdis, Medeiros-Domingo, Gaertner-Rommel, Kast, Enseleit, Bode, Klingel, Kandolf, Renois, Andreoletti, Akdis, Milting, Lüscher, Brunckhorst, Saguner, Duru: Myocardial expression profiles of candidate molecules in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia compared to those with dilated cardiomyopathy and healthy controls. dans Heart rhythm 2016
Mouse (Murine) Polyclonal Plakophilin 2 Primary Antibody pour ELISA, WB - ABIN4345997
Christensen, Benn, Tybjaerg-Hansen, Haunso, Svendsen: Missense variants in plakophilin-2 in arrhythmogenic right ventricular cardiomyopathy patients--disease-causing or innocent bystanders? dans Cardiology 2010
Mouse (Murine) Monoclonal Plakophilin 2 Primary Antibody pour IHC (p), IP - ABIN1169700
Sobolik-Delmaire, Reddy, Pashaj, Roberts, Wahl: Plakophilin-1 localizes to the nucleus and interacts with single-stranded DNA. dans The Journal of investigative dermatology 2010
Our study describes various clinical parameters in ACM patients and a recessive plakophilin 2 mutation after a limited PKP2 gene sequencing.
Authors show that the PKP2 gene encoding the desmosomal protein Plakophilin-2 is a novel direct transcriptional target of Wnt (Montrer WNT2 Anticorps)/beta-catenin (Montrer CTNNB1 Anticorps) in normal and colon cancer-associated fibroblasts.
Human iPSC line was generated from patient-specific adipose tissue-derived mesenchymal multipotent stromal cells carrying two mutations in plakophilin-2 (PKP2) gene using non-integrative reprogramming method.
The rare incidence of PKP2 mutation in sudden unexplained noctural death syndrome (SUNDS, 1%) supports the previous viewpoint that SUNDS is most likely an allelic disorder as Brugada syndrome
Up-regulation of plakophilin-2 (PKP2) is correlated with the progression of glioma. Study uncovers a potential role for PKP2 in the pathogenic process of glioma, suggesting that PKP2 may be a promising therapeutic target of glioma.
a novel homozygous Plakophilin 2-gene mutation has a role in advanced cardiomyopathy
An intronic mutation of c.2577+1G>T in the PKP2 gene causes a nonsyndromic form of Arrhythmogenic Right Ventricular Cardiomyopathy without cutaneous Involvements.
Data show that fetal pMSCs (mesenchymal stromal cells) expressing the highest levels of desmoglein 2 (Montrer DSG2 Anticorps), desmocollin 3 (Montrer DSC2 Anticorps) and plakophilin 2, followed by maternal pMSCs, while bmMSCs expressed the lowest levels.
Results show the involvement of plakophilin 2 protein (PKP2) in two siblings with severe cardiomyopathy with ventricular non compaction.
PKP2 c.419C>T did not associate with heart failure, arrhythmias, or premature death, with ARVC or HCM/DCM, or with effects in vitro, suggesting that this is not a disease-causing variant.
This study found that the PKP2 gene showed a downregulated profile only in the Inflexible when compared to the Light ethanol drinkers group.
Data indicate that lack of plakophilin-2 (PKP2) can cause arrhythmia in a structurally normal heart.
Results demonstrated that truncated PKP2 provokes arrhythmogenic cardiomyopathy in the absence of fibro-fatty replacement in the mouse.
PKP2 deficiency leads to suppression of the E2F1 (Montrer E2F1 Anticorps) pathway and hypermethylation of CPG sites at miR (Montrer MLXIP Anticorps)-184 promoter.
Plakophilin-2 loss promotes TGF-beta1 (Montrer TGFB1 Anticorps)/p38 MAPK (Montrer MAPK14 Anticorps)-dependent fibrotic gene expression in cardiomyocytes.
Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype.
PKP2 haploinsufficiency leads to I(Na) deficit in murine hearts which may contribute to generation and/or maintenance of arrhythmias.
plakophilin 2 is important for the assembly of junctional proteins and represents an essential morphogenic factor and architectural component of the heart
By providing an extra link between the cadherin-catenin complex and intermediate filaments, the binding of alphaT-catenin to plakophilin-2 is proposed to be a means of modulating and strengthening cell-cell adhesion between cardiac muscle cells.
PKP2 increased connexin 43 (Montrer GJA1 Anticorps), a gap junction protein, while its knockdown inhibited embryo implantation in mice.
This gene encodes a member of the arm-repeat (armadillo) and plakophilin gene families. Plakophilin proteins contain numerous armadillo repeats, localize to cell desmosomes and nuclei, and participate in linking cadherins to intermediate filaments in the cytoskeleton. This gene product may regulate the signaling activity of beta-catenin. Two alternately spliced transcripts encoding two protein isoforms have been identified. A processed pseudogene with high similarity to this locus has been mapped to chromosome 12p13.