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https://www.readbyqxmd.com/read/27902996/pemphigus-pathogenesis-to-treatment
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/27769725/structural-and-molecular-pathology-of%C3%A2-the%C3%A2-atrium-in-boxer-arrhythmogenic-right-ventricular-cardiomyopathy
#8
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...
October 18, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/27761164/the-genetic-background-of-arrhythmogenic-right-ventricular-cardiomyopathy
#9
Seiko Ohno
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by degeneration of the right ventricle and ventricular tachycardia originating from the right ventricle. Additionally, the disease is an inherited cardiomyopathy that mainly follows the autosomal dominant pattern. More than 10 genes have been reported as causative genes for ARVC, and more than half of ARVC patients carry mutations in desmosome related genes. The desmosome is one of the structures involved in cell adhesion and its disruption leads to various diseases, including a skin disease called pemphigus...
October 2016: Journal of Arrhythmia
https://www.readbyqxmd.com/read/27750289/pemphigus-autoantibodies-induce-blistering-in-human-conjunctiva
#10
Franziska Vielmuth, Vera Rötzer, Eva Hartlieb, Christoph Hirneiß, Jens Waschke, Volker Spindler
Purpose: The autoimmune blistering skin disease pemphigus vulgaris (PV) is caused by autoantibodies against desmosomal adhesion molecules. Patients may suffer conjunctival involvement, yet the underlying mechanisms are largely unclear. We characterized human and murine conjunctiva with respect to the PV autoantigens, and evaluated the effects and mechanisms of PV autoantibodies applied to human conjunctiva ex vivo. Methods: We obtained human conjunctiva specimens from surgical explants and established a short-term culture model to study the alterations induced by antibody fractions of PV patients (PV-IgG)...
August 1, 2016: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/27736720/genetic-basis-of-dilated-cardiomyopathy
#11
Alexandra Pérez-Serra, Rocio Toro, Georgia Sarquella-Brugada, David de Gonzalo-Calvo, Sergi Cesar, Esther Carro, Vicenta Llorente-Cortes, Anna Iglesias, Josep Brugada, Ramon Brugada, Oscar Campuzano
Dilated cardiomyopathy is a rare cardiac disease characterized by left ventricular dilatation and systolic dysfunction leading to heart failure and sudden cardiac death. Currently, despite several conditions have been reported as aetiologies of the disease, a large number of cases remain classified as idiopathic. Recent studies determine that nearly 60% of cases are inherited, therefore due to a genetic cause. Progressive technological advances in genetic analysis have identified over 60 genes associated with this entity, being TTN the main gene, so far...
December 1, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27736270/ultrastructural-changes-in-epithelial-cells-of-rats-exposed-to-low-concentration-of-hydrogen-sulfide-for-50-days
#12
Defne Yalçın Yeler, Murat Aydın, Peyami Turgay Hocaoğlu, Melike Koraltan, Hakan Özdemir, Tuğba Kotil, Mehmet Gül
Hydrogen sulfide (H2S) and other volatile sulfur compounds (VSCs) appear mainly in the oral air of patients with halitosis. It seems that VSCs are directly involved in the pathogenesis of gingival diseases. In previous studies, short-term (7 hours-4 days), high concentrations (5-400 ppm) of H2S applications on periodontal tissues have been evaluated in a culture medium. The aim of the present study was to investigate the potential effects of lower (equivalent to halitosis) concentrations of H2S on rat gingival tissue for longer-term inhalation...
November 2016: Ultrastructural Pathology
https://www.readbyqxmd.com/read/27715074/-arrhythmogenic-left-ventricular-cardiomyopathy
#13
Štěpán Havránek, Tomáš Paleček, Petr Kuchynka, Ivana Vítková
Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a rare condition characterised by progressive fibrofatty replacement of the myocardium of the left ventricle in combination with arrhythmias of left ventricular origin. ALVC has been linked to autosomal dominant mutations of genes encoding desmosomal proteins, similarly to the classic arrhythmogenic right ventricular cardiomyopathy with which it also shares pathological and prognostic features. It seems that isolated left or right ventricular abnormalities represent two extremes of the spectrum of clinical manifestations of a single disease: arrhythmogenic cardiomyopathy...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27703000/palmitoylation-of-desmoglein-2-is-a-regulator-of-assembly-dynamics-and-protein-turnover
#14
Brett J Roberts, Robert A Svoboda, Andrew M Overmiller, Joshua D Lewis, Andrew P Kowalczyk, My G Mahoney, Keith R Johnson, James K Wahl
Desmosomes are prominent adhesive junctions present between many epithelial cells as well as cardiomyocytes. The mechanisms controlling desmosome assembly and remodeling in epithelial and cardiac tissue are poorly understood. We recently identified protein palmitoylation as a mechanism regulating desmosome dynamics. In this study, we have focused on the palmitoylation of the desmosomal cadherin desmoglein-2 (Dsg2) and characterized the role that palmitoylation of Dsg2 plays in its localization and stability in cultured cells...
November 25, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27702552/the-effect-of-culture-medium-and-carrier-on-explant-culture-of-human-limbal-epithelium-a-comparison-of-ultrastructure-keratin-profile-and-gene-expression
#15
Meeta Pathak, O K Olstad, Liv Drolsum, Morten C Moe, Natalia Smorodinova, Sarka Kalasova, Katerina Jirsova, Bjørn Nicolaissen, Agate Noer
Patients with limbal stem cell deficiency (LSCD) often experience pain and photophobia due to recurrent epithelial defects and chronic inflammation of the cornea. Successfully restoring a healthy corneal surface in these patients by transplantation of ex vivo expanded human limbal epithelial cells (LECs) may alleviate these symptoms and significantly improve their quality of life. The clinical outcome of transplantation is known to be influenced by the quality of transplanted cells. Presently, several different protocols for cultivation and transplantation of LECs are in use...
October 1, 2016: Experimental Eye Research
https://www.readbyqxmd.com/read/27624909/cadherin-tales-regulation-of-cadherin-function-by-endocytic-membrane-trafficking
#16
REVIEW
Chantel M Cadwell, Wenji Su, Andrew P Kowalczyk
Cadherins are the primary adhesion molecules in adherens junctions and desmosomes and play essential roles in embryonic development. Although significant progress has been made in understanding cadherin structure and function, we lack a clear vision of how cells confer plasticity upon adhesive junctions to allow for cellular rearrangements during development, wound healing and metastasis. Endocytic membrane trafficking has emerged as a fundamental mechanism by which cells confer a dynamic state to adhesive junctions...
September 14, 2016: Traffic
https://www.readbyqxmd.com/read/27624852/exploring-beyond-the-oral-mucosa-in-patients-affected-with-autoimmune-blistering-diseases-the-importance-of-endoscopic-procedures
#17
A España, S Fernandez
Autoimmune blistering diseases (AIBD) comprise several entities characterized by the presence of autoantibodies targeted against structural proteins either in desmosomes or in the dermoepidermal junction of polystratified squamous epithelium. Patients develop blisters, erosions in cutaneous surfaces or mucosas. Diagnosis is based on the characteristic mucocutaneous lesions, the typical findings on histological studies and direct immunofluorescence assays, and the presence of specific autoantibodies against the epidermal antigens...
September 14, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27623697/ultrastructural-ssrdna-sequencing-of-myxobolus-prochilodus-and-myxobolus-porofilus-and-details-of-the-interaction-with-the-host-prochilodus-lineatus
#18
Suellen A Zatti, Sarah Arana, Antônio A M Maia, Edson A Adriano
Myxobolus prochilodus and Myxobolus porofilus are parasites of Prochilodus lineatus, an economically important South American fish found in La Plata and Paraiba do Sul river basins. This study focusing on parasite-host interaction provides an ultrastructural and phylogenetic analysis, the latter based on ssrDNA sequencing of these parasites respectively infecting the gill filaments and fins of P. lineatus taken from the Mogi Guaçu River, São Paulo, Brazil. A total of 13 adult specimens were examined in this study...
September 14, 2016: Parasitology Research
https://www.readbyqxmd.com/read/27617087/arrhythmogenic-cardiomyopathy-electrical-and-structural-phenotypes
#19
Deniz Akdis, Corinna Brunckhorst, Firat Duru, Ardan M Saguner
This overview gives an update on the molecular mechanisms, clinical manifestations, diagnosis and therapy of arrhythmogenic cardiomyopathy (ACM). ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Three subtypes have been proposed: the classical right-dominant subtype generally referred to as ARVC/D, biventricular forms with early biventricular involvement and left-dominant subtypes with predominant LV involvement. Typical symptoms include palpitations, arrhythmic (pre)syncope and sudden cardiac arrest due to ventricular arrhythmias, which typically occur in athletes...
August 2016: Arrhythmia & Electrophysiology Review
https://www.readbyqxmd.com/read/27611344/daughters-of-the-enamel-organ-development-fate-and-function-of-the-stratum-intermedium-stellate-reticulum-and-outer-enamel-epithelium
#20
Hui Liu, Xiulin Yan, Mirali Pandya, Xianghong Luan, Thomas G H Diekwisch
The tooth enamel organ (EO) is a complex epithelial cell assembly involved in multiple aspects of tooth development, including amelogenesis. The present study focuses on the role of the nonameloblast layers of the EO, the stratum intermedium, the stellate reticulum, and the outer enamel epithelium (OEE). The secretory stage stratum intermedium was distinguished by p63-positive epithelial stem cell marks, highly specific alkaline phosphatase labeling, as well as multiple desmosomes and gap junctions. At the location of the presecretory stage stellate reticulum, the pre-eruption EO prominently featured the papillary layer (PL) as a keratin immunopositive network of epithelial strands between tooth crowns and oral epithelium...
September 9, 2016: Stem Cells and Development
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