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https://www.readbyqxmd.com/read/28102477/potential-new-mechanisms-of-pro-arrhythmia-in-arrhythmogenic-cardiomyopathy-focus-on-calcium-sensitive-pathways
#1
REVIEW
C J M van Opbergen, M Delmar, T A B van Veen
Arrhythmogenic cardiomyopathy, or its most well-known subform arrhythmogenic right ventricular cardiomyopathy (ARVC), is a cardiac disease mainly characterised by a gradual replacement of the myocardial mass by fibrous and fatty tissue, leading to dilatation of the ventricular wall, arrhythmias and progression towards heart failure. ARVC is commonly regarded as a disease of the intercalated disk in which mutations in desmosomal proteins are an important causative factor. Interestingly, the Dutch founder mutation PLN R14Del has been identified to play an additional, and major, role in ARVC patients within the Netherlands...
January 19, 2017: Netherlands Heart Journal
https://www.readbyqxmd.com/read/28096266/desmosomes-and-intermediate-filaments-their-consequences-for-tissue-mechanics
#2
Mechthild Hatzfeld, René Keil, Thomas M Magin
Adherens junctions (AJs) and desmosomes connect the actin and keratin filament networks of adjacent cells into a mechanical unit. Whereas AJs function in mechanosensing and in transducing mechanical forces between the plasma membrane and the actomyosin cytoskeleton, desmosomes and intermediate filaments (IFs) provide mechanical stability required to maintain tissue architecture and integrity when the tissues are exposed to mechanical stress. Desmosomes are essential for stable intercellular cohesion, whereas keratins determine cell mechanics but are not involved in generating tension...
January 17, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28078185/cxcl11-expression-by-keratinocytes-occurs-transiently-between-reaching-confluence-and-cellular-compaction
#3
Arthur C Huen, Archana Marathi, Peter K Nam, Alan Wells
Objective: To investigate whether differentiation or cellular confluence is responsible for CXCL11 expression patterns in re-epithelialization. Approach:In vitro model systems of re-epithelialization using the HaCaT keratinocyte cell line were utilized in monitoring expression of differentiation markers, including desmoplakin and various cytokeratins while evaluating for an association with chemokine CXCL11 expression. Results: CXCL11 expression was elevated in sparse culture with peak expression near the time of confluence...
December 1, 2016: Advances in Wound Care
https://www.readbyqxmd.com/read/28073072/life-cycle-of-blastocrithidia-papi-sp-n-kinetoplastea-trypanosomatidae-in-pyrrhocoris-apterus-hemiptera-pyrrhocoridae
#4
Alexander O Frolov, Marina N Malysheva, Anna I Ganyukova, Vyacheslav Yurchenko, Alexei Y Kostygov
Blastocrithidia papi sp. n. is a cyst-forming trypanosomatid parasitizing firebugs (Pyrrhocoris apterus). It is a member of the Blastocrithidia clade and a very close relative of B. largi, to which it is almost identical through its SSU rRNA gene sequence. However, considering the SL RNA gene these two species represent quite distinct, not even related typing units. Morphological analysis of the new species revealed peculiar or even unique features, which may be useful for future taxonomic revision of the genus Blastocrithidia...
November 16, 2016: European Journal of Protistology
https://www.readbyqxmd.com/read/28069705/multilevel-analyses-of-scn5a-mutations-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-suggest-non-canonical-mechanisms-for-disease-pathogenesis
#5
Anneline S J M Te Riele, Esperanza Agullo-Pascual, Cynthia A James, Alejandra Leo-Macias, Marina Cerrone, Mingliang Zhang, Xianming Lin, Bin Lin, Nara L Sobreira, Nuria Amat-Alarcon, Roos F Marsman, Brittney Murray, Crystal Tichnell, Jeroen F van der Heijden, Dennis Dooijes, Toon A B van Veen, Harikrishna Tandri, Steven J Fowler, Richard N W Hauer, Gordon Tomaselli, Maarten P van den Berg, Matthew R G Taylor, Francesca Brun, Gianfranco Sinagra, Arthur A M Wilde, Luisa Mestroni, Connie R Bezzina, Hugh Calkins, J Peter van Tintelen, Lei Bu, Mario Delmar, Daniel P Judge
AIMS: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is often associated with desmosomal mutations. Recent studies suggest an interaction between the desmosome and sodium channel protein Nav1.5. We aimed to determine the prevalence and biophysical properties of mutations in SCN5A (the gene encoding Nav1.5) in ARVD/C. METHODS AND RESULTS: We performed whole-exome sequencing in six ARVD/C patients (33% male, 38.2 ± 12.1 years) without a desmosomal mutation...
January 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28069669/refining-the-molecular-organization-of-the-cardiac-intercalated-disc
#6
REVIEW
Sarah H Vermij, Hugues Abriel, Toon A B van Veen
This review presents an extensively integrated model of the cardiac intercalated disc (ID), a highly orchestrated structure that connects adjacent cardiomyocytes. Classically, three main structures are distinguished: gap junctions (GJs) metabolically and electrically connect cytoplasm of adjacent cardiomyocytes; adherens junctions (AJs) connect the actin cytoskeleton of adjacent cells; and desmosomes function as cell anchors and connect intermediate filaments. Furthermore, ion channels reside in the ID. Mutations in ID proteins have been associated with cardiac arrhythmias such as Brugada syndrome and arrhythmogenic cardiomyopathy...
January 8, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28069601/comprehensive-multi-modality-imaging-approach-in-arrhythmogenic-cardiomyopathy-an-expert-consensus-document-of-the-european-association-of-cardiovascular-imaging
#7
Kristina H Haugaa, Cristina Basso, Luigi P Badano, Chiara Bucciarelli-Ducci, Nuno Cardim, Oliver Gaemperli, Maurizio Galderisi, Gilbert Habib, Juhani Knuuti, Patrizio Lancellotti, William McKenna, Danilo Neglia, Bogdan A Popescu, Thor Edvardsen
Arrhythmogenic cardiomyopathy (AC) is a progressive disease with high risk of life-threatening ventricular arrhythmias. A genetic mutation is found in up to 50-60% of probands, mostly affecting desmosomal genes. Diagnosis of AC is made by a combination of data from different modalities including imaging, electrocardiogram, Holter monitoring, family history, genetic testing, and tissue properties. Being a progressive cardiomyopathy, repeated cardiac imaging is needed in AC patients. Repeated imaging is important also for risk assessment of ventricular arrhythmias...
January 9, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28066800/cd36-deficiency-impairs-the-small-intestinal-barrier-and-induces-subclinical-inflammation-in-mice
#8
Vincenza Cifarelli, Stoyan Ivanov, Yan Xie, Ni-Huiping Son, Brian T Saunders, Terri A Pietka, Trevor M Shew, Jun Yoshino, Sinju Sundaresan, Nicholas O Davidson, Ira J Goldberg, Andrew E Gelman, Bernd H Zinselmeyer, Gwendalyn J Randolph, Nada A Abumrad
BACKGROUND & AIMS: CD36 has immuno-metabolic actions and is abundant in the small intestine on epithelial, endothelial and immune cells. We examined the role of CD36 in gut homeostasis using mice null for CD36 (CD36KO) and with CD36 deletion specific to enterocytes (Ent-CD36KO) or endothelial cells (EC-CD36KO). METHODS: Intestinal morphology was evaluated using immunohistochemistry and electron microscopy (EM). Intestinal inflammation was determined from neutrophil infiltration and expression of cytokines, toll-like receptors and COX-2...
January 2017: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28052997/human-three-dimensional-endometrial-epithelial-cell-model-to-study-host-interactions-with-vaginal-bacteria-and-neisseria-gonorrhoeae
#9
Paweł Łaniewski, Adriana Gomez, Geoffrey Hire, Magdalene So, Melissa M Herbst-Kralovetz
Colonization of the endometrium by pathogenic bacteria, ascending from the lower female reproductive tract (FRT), is associated with many gynecologic and obstetric health complications. To study these host-microbe interactions in vitro, we developed a human three-dimensional (3-D) endometrial epithelial cell (EEC) model using the HEC-1A cell line and rotating wall vessel (RWV) bioreactor technology. Our model, composed of 3-D EEC aggregates, recapitulates several functional/structural characteristics of human endometrial epithelial tissue including cell differentiation, junctional complexes/desmosomes, microvilli, membrane-associated mucins and Toll-like receptors (TLRs)...
January 4, 2017: Infection and Immunity
https://www.readbyqxmd.com/read/28049646/intermediate-filaments-and-the-plasma-membrane
#10
REVIEW
Jonathan C R Jones, Chen Yuan Kam, Robert M Harmon, Alexandra V Woychek, Susan B Hopkinson, Kathleen J Green
A variety of intermediate filament (IF) types show intricate association with plasma membrane proteins, including receptors and adhesion molecules. The molecular basis of linkage of IFs to desmosomes at sites of cell-cell interaction and hemidesmosomes at sites of cell-matrix adhesion has been elucidated and involves IF-associated proteins. However, IFs also interact with focal adhesions and cell-surface molecules, including dystroglycan. Through such membrane interactions, it is well accepted that IFs play important roles in the establishment and maintenance of tissue integrity...
January 3, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28024684/non-pathogenic-pemphigus-foliaceus-pf-igg-acts-synergistically-with-a-directly-pathogenic-pf-igg-to-increase-blistering-by-p38mapk-dependent-desmoglein-1-clustering
#11
Kenji Yoshida, Ken Ishii, Atsushi Shimizu, Mariko Yokouchi, Masayuki Amagai, Ken Shiraishi, Yuji Shirakata, John R Stanley, Akira Ishiko
BACKGROUND: Pemphigus foliaceus (PF) is an autoimmune blistering disease caused by autoantibodies (Abs) against desmoglein 1 (Dsg1). PF sera contain polyclonal Abs which are heterogeneous mixture of both pathogenic and non-pathogenic Abs, as shown by isolation of monoclonal Abs (mAbs). OBJECTIVE: To investigate how pathogenic and non-pathogenic anti-Dsg1 Abs contribute to blister formation in PF. METHODS: Using organ-cultured human skin, we compared the effect of a single pathogenic anti-Dsg1 IgG mAb, a single non-pathogenic anti-Dsg1 IgG mAb, and their mixture on blister formation as analyzed by histology, subcellular localization of IgG deposits and desmosomal proteins by confocal microscopy, and desmosomal structure by electron microscopy...
December 12, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/27999452/microrna-regulation-of-endothelial-junction-proteins-and-clinical-consequence
#12
REVIEW
Yugang Zhuang, Hu Peng, Victoria Mastej, Weiguo Chen
Cellular junctions play a critical role in structural connection and signal communication between cells in various tissues. Although there are structural and functional varieties, cellular junctions include tight junctions, adherens junctions, focal adhesion junctions, and tissue specific junctions such as PECAM-1 junctions in endothelial cells (EC), desmosomes in epithelial cells, and hemidesmosomes in EC. Cellular junction dysfunction and deterioration are indicative of clinical diseases. MicroRNAs (miRNA) are ~20 nucleotide, noncoding RNAs that play an important role in posttranscriptional regulation for almost all genes...
2016: Mediators of Inflammation
https://www.readbyqxmd.com/read/27982550/desmosomes-and-corneodesmosomes-and-their-relevance-to-genetic-skin-diseases
#13
Akemi Ishida-Yamamoto, Mari Kishibe, Masaru Honma
Desmosomes are critical intercellular junctions between keratinocytes in the living cell layers of the epidermis. When the cells are differentiated and become cornified cells, desmosomes are transformed into corneodesmosomes. Distribution patterns of corneodesmosomes change with cell development. Namely, in the lower stratum corneum, corneodesmosomes are seen all around the cell membrane, but in the upper layers, they are only found at the edges of the flattened cells. Recently, it has been proposed that tight junction-derived structures are involved in this unique distribution of corneodesmosomes by protecting corneodesmosome degradation from proteases...
December 16, 2016: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/27902996/pemphigus-pathogenesis-to-treatment
#14
Christopher DiMarco
Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. These lesions correlate histologically with splits forming in the epidermis, leaving a blister roof composed of a few cell layers...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27889952/effects-of-different-doses-of-2-3-dimercaptosuccinic-acid-modified-fe2-o3-nanoparticles-on-intercalated-discs-in-engineered-cardiac-tissues
#15
Yongchao Mou, Shuanghong Lv, Fei Xiong, Yao Han, Yuwei Zhao, Junjie Li, Ning Gu, Jin Zhou
Although iron oxide nanoparticles (IRONs) were applied in clinical magnetic resonance imaging in vivo and magnetic tissue engineering in vitro widely, the underlying effects of IRONs on the development of cardiomyocytes especially the intercellular junctions, intercalated discs (IDs), remain an unknown issue. Given the critical role of three-dimensional (3D) engineered cardiac tissues (ECTs) in evaluation of nanoparticles toxicology, it remained necessary to understand the effects of IRONs on IDs assembly of cardiomyocytes in 3D environment...
November 27, 2016: Journal of Biomedical Materials Research. Part B, Applied Biomaterials
https://www.readbyqxmd.com/read/27882603/establishment-and-characterization-of-a-cell-population-derived-from-a-dentigerous-cyst
#16
Marcos A Muñiz-Lino, Mariana Rodríguez-Vázquez, Bibiana Chávez-Munguía, Josué Z Ortiz-García, Lorena González-López, Fidel C Hernández-Hernández, Carlos Licéaga-Escalera, Alejandro García-Muñoz, Mario A Rodríguez
BACKGROUND: Dentigerous cyst occurs in approximately 20% of jaw cysts, being the second major common odontogenic cyst, after radicular cyst. This oral lesion has the ability to destroy maxillary bones and could be the origin of several odontogenic tumors. However, molecules implicated in its pathogenesis as well as those involved in its neoplastic transformation remain unknown. Here, we established a cell population derived from a dentigerous cyst as an in vitro model for the study of this oral lesion...
November 24, 2016: Journal of Oral Pathology & Medicine
https://www.readbyqxmd.com/read/27855808/right-ventricular-imaging-and-computer-simulation-for-electromechanical-substrate-characterization-in-arrhythmogenic-right-ventricular-cardiomyopathy
#17
Thomas P Mast, Arco J Teske, John Walmsley, Jeroen F van der Heijden, René van Es, Frits W Prinzen, Tammo Delhaas, Toon A van Veen, Peter Loh, Pieter A Doevendans, Maarten J Cramer, Joost Lumens
BACKGROUND: Previous studies suggested that electrical abnormalities precede overt structural disease in arrhythmogenic right ventricular cardiomyopathy (ARVC). Abnormal RV deformation has been reported in early ARVC without structural abnormalities. The pathophysiological mechanisms underlying these abnormalities remain unknown. OBJECTIVES: The authors used imaging and computer simulation to differentiate electrical from mechanical tissue substrates among ARVC clinical stages...
November 15, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27834139/microrna-130a-regulation-of-desmocollin-2-in-a-novel-model-of-arrhythmogenic-cardiomyopathy
#18
Stefan R Mazurek, Tyler Calway, Cynthia Harmon, Priyanka Farrell, Gene H Kim
BACKGROUND: MicroRNAs are small noncoding RNA molecules that play a critical role in regulating physiological and disease processes. Recent studies have now recognized microRNAs as an important player in cardiac arrhythmogenesis. Molecular insight into arrhythmogenic cardiomyopathy (AC) has primarily focused on mutations in desmosome proteins. To our knowledge, models of AC due to microRNA dysregulation have not been reported. Previously, we reported on miR-130a mediated down-regulation of Connexin43...
November 9, 2016: MicroRNA
https://www.readbyqxmd.com/read/27792691/-hla-class-ii-in-mexican-patients-with-pemphigus-vulgaris-shared-epitope-for-autoimmunity
#19
Lucía Rangel-Gamboa, María Elisa Vega-Memije, Víctor Acuña-Alonzo, Julio Granados-Arriola
INTRODUCTION: Pemphigus is an autoimmune blistering disease of skin and mucous membranes characterized by presence of IgG antibodies against desmoglein 3, and 1. Desmoglein 3 and 1 are presented in pemphigus vulgaris and pemphigus foliaceous, respectively. Desmoglein are transmembrane proteins that form part of cellular junctions called desmosomes. Major histocompatibility complex class II molecules have been related to autoimmune disease; in pemphigus vulgaris, different human lymphocyte antigens (HLA) were associated among different ethnic groups, such as HLA-DR4, HLA-DR14, and HLA-DR1...
September 2016: Gaceta Médica de México
https://www.readbyqxmd.com/read/27776163/potential-use-of-autologous-renal-cells-from-diseased-kidneys-for-the-treatment-of-renal-failure
#20
Sunil K George, Mehran Abolbashari, John D Jackson, Tamer Aboushwareb, Anthony Atala, James J Yoo
Chronic kidney disease (CKD) occurs when certain conditions cause the kidneys to gradually lose function. For patients with CKD, renal transplantation is the only treatment option that restores kidney function. In this study, we evaluated primary renal cells obtained from diseased kidneys to determine whether their normal phenotypic and functional characteristics are retained, and could be used for cell therapy. Primary renal cells isolated from both normal kidneys (NK) and diseased kidneys (CKD) showed similar phenotypic characteristics and growth kinetics...
2016: PloS One
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