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Plakophilin

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https://www.readbyqxmd.com/read/28169297/comparative-influenza-protein-interactomes-identify-the-role-of-plakophilin-2-in-virus-restriction
#1
Lingyan Wang, Bishi Fu, Wenjun Li, Girish Patil, Lin Liu, Martin E Dorf, Shitao Li
Cellular protein interaction networks are integral to host defence and immune signalling pathways, which are often hijacked by viruses via protein interactions. However, the comparative virus-host protein interaction networks and how these networks control host immunity and viral infection remain to be elucidated. Here, we mapped protein interactomes between human host and several influenza A viruses (IAV). Comparative analyses of the interactomes identified common and unique interaction patterns regulating innate immunity and viral infection...
February 7, 2017: Nature Communications
https://www.readbyqxmd.com/read/28154962/expression-of-desmoglein-2-desmocollin-3-and-plakophilin-2-in-placenta-and-bone-marrow-derived-mesenchymal-stromal-cells
#2
Melanie L Hart, Elisa Rusch, Marvin Kaupp, Kay Nieselt, Wilhelm K Aicher
Many controversial results exist when comparing mesenchymal stromal cells (MSCs) derived from different sources. Reasons include not only variables in tissue origin, but also methods of cell preparation or choice of expansion media which can strongly influence the expression and hence, function of the cells. In this short report we aimed to investigate the expression of the cell anchoring proteins desmoglein 2, desmocollin 3 and plakophilin 2 in early passage placenta-derived MSCs of fetal (fetal pMSCs) and maternal (maternal pMSCs) origins versus adult bone marrow-derived MSCs (bmMSCs) that were expanded and cultured under the same good manufacturing practice (GMP) conditions...
February 2, 2017: Stem Cell Reviews
https://www.readbyqxmd.com/read/28124385/up-regulation-of-plakophilin-2-is-correlated-with-the-progression-of-glioma
#3
Degeng Zhang, Yuxia Qian, Xiaoxing Liu, Hong Yu, Niangao Zhao, Zhengdong Wu
Glioma is the most common type of primary brain tumor in the CNS. Due to its poor prognosis and high mortality rates, it is urgent to find out more effective therapies. Plakophilin-2 (PKP2) is a widespread desmosomal plaque protein. Recently, the important roles of PKP2 in the proliferation and migration of cancer cells and tumor progression has been shown. However, the expression and potential function of PKP2 in glioma was still unclear. In this study, we demonstrated that PKP2 protein expression level was increased in glioma tissues compared with normal brain tissues, and its level was significantly associated with the Ki-67 expression and WHO grade by Western blot analysis and immunohistochemistry...
January 26, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/27769725/structural-and-molecular-pathology-of%C3%A2-the%C3%A2-atrium-in-boxer-arrhythmogenic-right-ventricular-cardiomyopathy
#4
J Vila, R Pariaut, N S Moïse, E M Oxford, P R Fox, C A Reynolds, C Saelinger
OBJECTIVE: To investigate the expression and distribution of desmosomal and gap junction proteins of the intercalated disc in the atria of boxers with arrhythmogenic right ventricular cardiomyopathy (ARVC). ANIMALS: Nineteen control dogs and 13 boxers with histopathologically confirmed ARVC. METHODS: Right and left atrial samples were examined using immunofluorescence and Western blots. The intercalated disc proteins investigated included total and phosphorylated connexin43 (Cx43 and pCx43), connexin45, connexin40, plakoglobin, plakophilin-2, desmoplakin, and N-cadherin...
February 2017: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/27572111/influence-of-genotype-on-structural-atrial-abnormalities-and-atrial-fibrillation-or-flutter-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#5
Mimount Bourfiss, Anneline S J M Te Riele, Thomas P Mast, Maarten J Cramer, Jeroen F VAN DER Heijden, Toon A B VAN Veen, Peter Loh, Dennis Dooijes, Richard N W Hauer, Birgitta K Velthuis
INTRODUCTION: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is associated with desmosomal mutations. Although desmosomal disruption affects both ventricles and atria, little is known about atrial involvement in ARVD/C. OBJECTIVE: To describe the extent and clinical significance of structural atrial involvement and atrial arrhythmias (AA) in ARVD/C stratified by genotype. METHODS: We included 71 patients who met ARVD/C Task Force Criteria and underwent cardiac magnetic resonance (CMR) imaging and molecular genetic analysis...
December 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27477171/keratinization-disorders-and-genetic-aspects-in-palmar-and-plantar-keratodermas
#6
Ewa Stypczyńska, Waldemar Placek, Barbara Zegarska, Rafał Czajkowski
Palmoplantar keratoderma (PPK) is a heterogeneous group of hereditary and acquired disorders characterized by abnormal thickening of the palms and soles. There are three clinical patterns: diffuse, focal, and punctuate. Palmoplantar keratodermas can be divided into the following functional subgroups: disturbed gene functions in structural proteins (keratins), cornified envelope (loricrin, transglutaminase), cohesion (plakophilin, desmoplakin, desmoglein 1), cell-to-cell communication (connexins) and transmembrane signal transduction (cathepsin C)...
June 2016: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/27470638/knockdown-of-plakophilin-2-downregulates-mir-184-through-cpg-hypermethylation-and-suppression-of-the-e2f1-pathway-and-leads-to-enhanced-adipogenesis-in-vitro
#7
Priyatansh Gurha, Xiaofan Chen, Raffaella Lombardi, James T Willerson, Ali J Marian
RATIONALE: PKP2, encoding plakophilin 2 (PKP2), is the most common causal gene for arrhythmogenic cardiomyopathy. OBJECTIVE: To characterize miRNA expression profile in PKP2-deficient cells. METHODS AND RESULTS: Control and PKP2-knockdown HL-1 (HL-1(Pkp2-shRNA)) cells were screened for 750 miRNAs using low-density microfluidic panels. Fifty-nine miRNAs were differentially expressed. MiR-184 was the most downregulated miRNA. Expression of miR-184 in the heart and cardiac myocyte was developmentally downregulated and was low in mature myocytes...
September 2, 2016: Circulation Research
https://www.readbyqxmd.com/read/27412010/molecular-disturbance-underlies-to-arrhythmogenic-cardiomyopathy-induced-by-transgene-content-age-and-exercise-in-a-truncated-pkp2-mouse-model
#8
Javier Moncayo-Arlandi, Eduard Guasch, Maria Sanz, Marta Casado, Nahuel Aquiles García, Lluis Mont, Marta Sitges, Ralph Knöll, Byambajav Buyandelger, Campuzano Oscar, Antonio Diez-Juan, Ramon Brugada
Arrhythmogenic cardiomyopathy (ACM) is a disorder characterized by a progressive ventricular myocardial replacement by fat and fibrosis which lead to ventricular arrhythmias and sudden cardiac death. Mutations in the desmosomal gene Plakophilin-2 (PKP2) accounts for >40% of all known mutations, generally causing a truncated protein. In a PKP2 truncated mouse model, we hypothesize that content of transgene, endurance training and aging will be determinant in disease progression. In addition, we investigated the molecular defects associated with the phenotype in this model...
July 12, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27375112/antagonistic-regulation-of-intercellular-cohesion-by-plakophilins-1-and-3
#9
René Keil, Katrin Rietscher, Mechthild Hatzfeld
Desmosomes are cell-cell adhesive structures essential for tissue integrity of the epidermis and the heart. Their constituents belong to multigene families giving rise to desmosomes of variable composition. So far, the functional significance of context-dependent composition in desmosome formation, dynamics, or stability during epidermal differentiation is incompletely understood. In this comparative study, we have uncovered unique and partially antagonistic functions of plakophilins 1 and 3 that are both expressed in the murine epidermis...
October 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27357287/multiple-regulatory-variants-located-in-cell-type-specific-enhancers-within-the-pkp2-locus-form-major-risk-and-protective-haplotypes-for-canine-atopic-dermatitis-in-german-shepherd-dogs
#10
Katarina Tengvall, Sergey Kozyrev, Marcin Kierczak, Kerstin Bergvall, Fabiana H G Farias, Brita Ardesjö-Lundgren, Mia Olsson, Eva Murén, Ragnvi Hagman, Tosso Leeb, Gerli Pielberg, Åke Hedhammar, Göran Andersson, Kerstin Lindblad-Toh
BACKGROUND: Canine atopic dermatitis (CAD) is a chronic inflammatory skin disease triggered by allergic reactions involving IgE antibodies directed towards environmental allergens. We previously identified a ~1.5 Mb locus on canine chromosome 27 associated with CAD in German shepherd dogs (GSDs). Fine-mapping indicated association closest to the PKP2 gene encoding plakophilin 2. RESULTS: Additional genotyping and association analyses in GSDs combined with control dogs from five breeds with low-risk for CAD revealed the top SNP 27:19,086,778 (p = 1...
2016: BMC Genetics
https://www.readbyqxmd.com/read/27122407/molecular-autopsy-of-desmosomal-protein-plakophilin-2-in-sudden-unexplained-nocturnal-death-syndrome
#11
Lei Huang, Shuangbo Tang, Longyun Peng, Yili Chen, Jianding Cheng
Plakophilin-2 (PKP2) variants could produce a phenotype of Brugada syndrome (BrS), which seems to be most likely the same allelic disorder as some sudden unexplained nocturnal death syndrome (SUNDS). All coding regions of PKP2 gene in 119 SUNDS victims were genetically screened using PCR and direct Sanger sequencing methods. Three novel mutations (p.Ala159Thr, p.Val200Val, and p.Gly265Glu), one novel rare polymorphism (p.Thr723Thr), and eight reported polymorphisms were identified. A compound mutation (p.Ala159Thr and p...
May 2016: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/27085656/brugada-syndrome-and-pkp2-evidences-and-uncertainties
#12
EDITORIAL
Oscar Campuzano, Anna Fernández-Falgueras, Anna Iglesias, Ramon Brugada
Common electrocardiographic patterns in Brugada Syndrome and Arrhythmogenic Cardiomyopathy have been reported despite phenotypic alterations during its clinical course. Recently, potentially pathogenic variants in the PKP2 gene, the most prevalent gene associated with Arrhythmogenic Cardiomyopathy, have been associated with Brugada Syndrome. In addition, in vitro studies demonstrated the interaction between plakophilin-2 and sodium channel, the most prevalent gene associated with Brugada Syndrome. All these facts reinforce the suggested overlapping between both entities but little is known about the pathophysiological mechanisms...
July 1, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27033150/growth-retardation-loss-of-desmosomal-adhesion-and-impaired-tight-junction-function-identify-a-unique-role-of-plakophilin-1-in%C3%A2-vivo
#13
Katrin Rietscher, Annika Wolf, Gerd Hause, Annekatrin Rother, René Keil, Thomas M Magin, Markus Glass, Carien M Niessen, Mechthild Hatzfeld
Desmosomes mediate strong intercellular adhesion through desmosomal cadherins that interact with intracellular linker proteins including plakophilins (PKPs) 1-3 to anchor the intermediate filaments. PKPs show overlapping but distinct expression patterns in the epidermis. So far, the contribution of individual PKPs in differentially regulating desmosome function is incompletely understood. To resolve the role of PKP1 we ablated the PKP1 gene. Here, we report that PKP1(-/-) mice were born at the expected mendelian ratio with reduced birth weight, but they otherwise appeared normal immediately after birth...
July 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27033120/new-partners-and-phosphorylation-sites-of-focal-adhesion-kinase-identified-by-mass-spectrometry
#14
Maria del Mar Masdeu, Beatriz G Armendáriz, Eduardo Soriano, Jesús Mariano Ureña, Ferran Burgaya
The regulation of focal adhesion kinase (FAK) involves phosphorylation and multiple interactions with other signaling proteins. Some of these pathways are relevant for nervous system functions such as branching, axonal guidance, and plasticity. In this study, we screened mouse brain to identify FAK-interactive proteins and phosphorylatable residues as a first step to address the neuronal functions of this kinase. Using mass spectrometry analysis, we identified new phosphorylated sites (Thr 952, Thr 1048, and Ser 1049), which lie in the FAT domain; and putative new partners for FAK, which include cytoskeletal proteins such as drebrin and MAP 6, adhesion regulators such as neurabin-2 and plakophilin 1, and synapse-associated proteins such as SynGAP and a NMDA receptor subunit...
July 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27015268/plakophilin-1-a-novel-wnt-signaling-regulator-is-critical-for-tooth-development-and-ameloblast-differentiation
#15
Kanako Miyazaki, Keigo Yoshizaki, Chieko Arai, Aya Yamada, Kan Saito, Masaki Ishikawa, Han Xue, Keita Funada, Naoto Haruyama, Yoshihiko Yamada, Satoshi Fukumoto, Ichiro Takahashi
Tooth morphogenesis is initiated by reciprocal interactions between the ectoderm and neural crest-derived mesenchyme, and the Wnt signaling pathway is involved in this process. We found that Plakophilin (PKP)1, which is associated with diseases such as ectodermal dysplasia/skin fragility syndrome, was highly expressed in teeth and skin, and was upregulated during tooth development. We hypothesized that PKP1 regulates Wnt signaling via its armadillo repeat domain in a manner similar to β-catenin. To determine its role in tooth development, we performed Pkp1 knockdown experiments using ex vivo organ cultures and cell cultures...
2016: PloS One
https://www.readbyqxmd.com/read/26995964/-the-effect-of-plakophilin-2-gene-mutations-on-activity-of-the-canonical-wnt-signaling-pathway
#16
A A Khudiakov, D A Kostina, A A Kostareva, A N Tomilin, A B Malashicheva
Plakophilin-2 is a desmosomal protein encoded by PKP2 gene. Desmosomal proteins are usually considered as structural proteins with the main function of maintaining intercellular interactions. Genetic studies revealed that mutations in desmosomal genes could lead to arrhythmogenic right ventricular cardiomyopathy, heart disease characterized by substitution of cardiomyocytes by adipose and fibrotic tissue predominantly in right ventricle. Wnt signaling pathway is one of the signal transduction pathways which could be involved in the formation of the pathology...
2015: Tsitologiia
https://www.readbyqxmd.com/read/26887364/-the-clinical-analysis-of-plakophilin-2-gene-mutation-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia
#17
Xin Liu, Wenling Liu, Dayi Hu, Li Zhang, Tiangang Zhu, Cuilan Li, Xiaoliang Qiu, Lei Li, Chengjun Guo, Xingpeng Liu, Lei Wang, Hua Zheng, Chunling Wang, Qing Diao, Dan Shi, Pingyun Zhan, Yuanming Deng, Kunshen Liu, Yi Wang, Baomin Liu, Hongming Liu, Xuguang Qin, Yanping Lan
OBJECTIVE: The purpose of this study was to screen genetic variations in plakophilin-2 (PKP2) gene in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) and investigate the differences in clinical features between mutation and no-mutation groups. METHODS: Thirty unrelated Chinese patients clinically diagnosed with ARVC/D and 50 healthy controls were included. Genomic DNA was isolated from peripheral blood samples. PCR and direct sequencing were used to detect variations in PKP2 gene...
December 2015: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/26879535/skin-needs-plakophilin-1
#18
Maybo Chiu, Zhuxiang Nie
No abstract text is available yet for this article.
January 2007: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/26877139/mechanisms-of-pulmonary-cyst-pathogenesis-in-birt-hogg-dube-syndrome-the-stretch-hypothesis
#19
REVIEW
John C Kennedy, Damir Khabibullin, Elizabeth P Henske
Loss-of-function mutations in the folliculin gene (FLCN) on chromosome 17p cause Birt-Hogg-Dube syndrome (BHD), which is associated with cystic lung disease. The risk of lung collapse (pneumothorax) in BHD patients is 50-fold higher than in the general population. The cystic lung disease in BHD is distinctive because the cysts tend to be basilar, subpleural and lentiform, differentiating BHD from most other cystic lung diseases. Recently, major advances in elucidating the primary functions of the folliculin protein have been made, including roles in mTOR and AMPK signaling via the interaction of FLCN with FNIP1/2, and cell-cell adhesion via the physical interaction of FLCN with plakophilin 4 (PKP4), an armadillo-repeat containing protein that interacts with E-cadherin and is a component of the adherens junctions...
April 2016: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/26858265/plakophilin-2-loss-promotes-tgf-%C3%AE-1-p38-mapk-dependent-fibrotic-gene-expression-in-cardiomyocytes
#20
Adi D Dubash, Chen Y Kam, Brian A Aguado, Dipal M Patel, Mario Delmar, Lonnie D Shea, Kathleen J Green
Members of the desmosome protein family are integral components of the cardiac area composita, a mixed junctional complex responsible for electromechanical coupling between cardiomyocytes. In this study, we provide evidence that loss of the desmosomal armadillo protein Plakophilin-2 (PKP2) in cardiomyocytes elevates transforming growth factor β1 (TGF-β1) and p38 mitogen-activated protein kinase (MAPK) signaling, which together coordinate a transcriptional program that results in increased expression of profibrotic genes...
February 15, 2016: Journal of Cell Biology
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