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https://www.readbyqxmd.com/read/28211055/pemphigus-a-pathomechanism-of-acantholysis
#1
REVIEW
Masutaka Furue, Takafumi Kadono
Autoantibodies to the desmosomal proteins desmoglein 1 and 3 cause pemphigus foliaceus and pemphigus vulgaris, which are characterised by keratinocyte dissociation (acantholysis) and intraepidermal blister formation. The passive transfer of pathogenic anti-desmoglein antibodies induces blisters in mice in vivo and the loss of keratinocyte adhesion in vitro. The pathogenetic mechanisms of acantholysis due to anti-desmoglein autoantibodies are not fully understood. However, recent studies have revealed that signalling-dependent and signalling-independent pathways are operative in the loss of cell adhesion...
February 17, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28197992/study-of-the-association-between-human-leukocyte-antigens-hla-and-pemphigus-vulgaris-in-brazilian-patients
#2
Julio M Gil, Raimar Weber, Claudia B Rosales, Helcio Rodrigues, Luiz U Sennes, Jorge Kalil, Azis Chagury, Ivan D Miziara
BACKGROUND: Pemphigus vulgaris is a mucocutaneous blistering autoimmune disease that manifests as painful blisters or erosions on the skin and/or mucosal surfaces. IgG autoantibodies target desmoglein, playing a major role in disease pathogenesis. Genetic predisposal to pemphigus vulgaris, especially the HLA DR and DQ alleles, has been known since the 1980s. The unique constitution of the Brazilian population favors exploratory genetic studies. METHODS: The study group included 51 patients with a confirmed diagnosis of pemphigus vulgaris from a tertiary hospital in Sao Paulo city, Sao Paulo, southeast Brazil...
February 15, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28183531/cardiomyocyte-hypertrophy-in-arrhythmogenic-cardiomyopathy
#3
Mustafa Gerçek, Muhammed Gerçek, Sebastian Kant, Sakine Simsekyilmaz, Astrid Kassner, Hendrik Milting, Elisa A Liehn, Rudolf E Leube, Claudia A Krusche
Arrhythmogenic cardiomyopathy (AC) is a hereditary disease leading to sudden cardiac death or heart failure. AC pathology is characterized by cardiomyocyte loss and replacement fibrosis. Our goal was to determine whether cardiomyocytes respond to AC progression by pathological hypertrophy. To this end, we examined tissue samples from AC patients with end-stage heart failure and tissue samples that were collected at different disease stages from desmoglein 2-mutant mice, a well characterized AC model. We find that cardiomyocyte diameters are significantly increased in right ventricles of AC patients...
February 6, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28164651/anti-desmoglein-1-and-3-autoantibody-levels-in-endemic-pemphigus-foliaceus-and-pemphigus-vulgaris-from-brazil
#4
Liana A Oliveira, Alfredo Marquart-Filho, Gerson Trevilato, Rodolfo P Timoteo, Maira Mukai, Ana M F Roselino, Maria L Petzl-Erler
BACKGROUND: Pemphigus is a group of autoimmune blistering diseases of which the major forms are pemphigus foliaceus (PF) and vulgaris (PV). In Brazil, PF occurs in an endemic form also known as fogo selvagem. The main autoantibody in PF is against desmoglein 1 (DSG1), while in PV the main antibody is anti-desmoglein 3 (DSG3), but often anti-DSG1 is also present. The aim of the present study was to analyze the levels of anti-DSG1 and antiDSG3 autoantibodies in Brazilian PF and PV patients, considering different stages of the disease for PF patients and comparing these levels to those of healthy individuals living in and outside the endemic regions...
July 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28154962/expression-of-desmoglein-2-desmocollin-3-and-plakophilin-2-in-placenta-and-bone-marrow-derived-mesenchymal-stromal-cells
#5
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/28131390/calpain-14-and-its-association-with-eosinophilic-esophagitis
#6
REVIEW
Vladislav A Litosh, Mark Rochman, Jeffrey K Rymer, Aleksey Porollo, Leah C Kottyan, Marc E Rothenberg
Calpains are a family of intracellular, calcium-dependent cysteine proteases involved in a variety of regulatory processes, including cytoskeletal dynamics, cell-cycle progression, signal transduction, gene expression, and apoptosis. These enzymes have been implicated in a number of disease processes, notably for this review involving eosinophilic tissue inflammation, such as eosinophilic esophagitis (EoE), a chronic inflammatory disorder triggered by allergic hypersensitivity to food and associated with genetic variants in calpain 14 (CAPN14)...
January 25, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28089115/correlation-of-antimuscarinic-acetylcholine-receptor-antibody-titers-and-antidesmoglein-antibody-titers-with-the-severity-of-disease-in-patients-with-pemphigus
#7
Manimegalai Jeyasekaran Dhanabhakya Lakshmi, Telanseri Jaykar Jaisankar, Medha Rajappa, Devinder Mohan Thappa, Laxmisha Chandrashekar, Dakshinamurthy Divyapriya, Malathi Munisamy, Gunaseelan Revathy
BACKGROUND: Acetylcholine receptor (AchR) antibody levels significantly correlate with disease severity at initial pemphigus diagnosis and during follow-up. However, it is not clear if they are just an epiphenomenon or a potential trigger of the known pathogenic process in pemphigus vulgaris. OBJECTIVE: We sought to assess the changes in anti-muscarinic (M3) AchR and anti-desmoglein (Dsg) antibody titers with therapy. METHODS: This was a hospital-based cohort study involving 45 patients with active pemphigus...
January 11, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28081338/new-immunohistochemical-markers-in-the-differential-diagnosisof-nonsmall-cell-lung-carcinoma
#8
Ferda Bir, Duygu Çeliker, Binnaz Fatma Evyapan, Arzu Yaren, Tamer Edirne
BACKGROUND/AIM: The aim of this study was to investigate Napsin-A, NTRK-1, NTRK-2, Desmoglein-3, and Desmocollin-3 in the differential diagnosis and prognosis of nonsmall cell lung cancer. MATERIALS AND METHODS: The expression of Napsin-A, NTRK-1, NTRK-2, and Desmoglein-3 was examined by immunohistochemistry in 50 squamous cell carcinomas and 50 adenocarcinomas. Desmocollin-3 was investigated in 29 squamous cell carcinoma and 29 adenocarcinoma cases. Associations between expression profiles of Napsin-A, NTRK-1, NTRK-2, Desmoglein-3, and Desmocollin-3 in lung cancers and clinicopathological variables were analyzed...
December 20, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28064226/salivary-samples-for-the-diagnosis-of-pemphigus-vulgaris-using-the-biochip-approach-a-pilot-study
#9
Irene Russo, Andrea Saponeri, Anna Michelotto, Mauro Alaibac
Pemphigus vulgaris (PV) is a rare autoimmune intraepithelial blistering skin disease characterized by the presence of circulating autoantibodies against desmoglein 3 (DSG3) and desmoglein 1 (DSG1), resulting in loss of the normal epithelial cell-to-cell adhesion, through a process called acantholysis. In recent years, a BIOCHIP-based indirect immunofluorescence technique for the determination of anti-DSG3 and anti-DSG1 autoantibodies has been described. Even though, the use of saliva anti-DSG3 and anti-DSG1 ELISA for the diagnosis of PV has been already reported, there are no studies concerning the utilization of saliva by the BIOCHIP approach...
2, 2017: In Vivo
https://www.readbyqxmd.com/read/28038887/prospective-studies-on-the-routine-use-of-a-novel-multivariant-enzyme-linked-immunosorbent-assay-for-the-diagnosis-of-autoimmune-bullous-diseases
#10
Nina van Beek, Cornelia Dähnrich, Nora Johannsen, Susanne Lemcke, Stephanie Goletz, Franziska Hübner, Giovanni Di Zenzo, Marian Dmochowski, Kossara Drenovska, Shamir Geller, Michael Horn, Cezary Kowalewski, Ljiljana Medenica, Dedee F Murrell, Aikaterini Patsatsi, Soner Uzun, Snejina Vassileva, Detlef Zillikens, Wolfgang Schlumberger, Enno Schmidt
BACKGROUND: Serologic diagnosis of autoimmune blistering disease (AIBD) usually follows a sophisticated multistep algorithm. OBJECTIVE: We sought validation of a multivariant enzyme-linked immunosorbent assay (ELISA) in the routine diagnosis of AIBD. METHODS: The multivariant ELISA comprising 6 recombinant immunodominant forms of major AIBD target antigens, ie, desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, and type VII collagen was applied in: (1) a cohort of well-characterized AIBD (n = 173) and control sera (n = 130), (2) a prospective multicenter study with 204 sera from patients with newly diagnosed AIBD with positive direct immunofluorescence microscopy, and (3) a prospective monocenter study with 292 consecutive sera from patients with clinical suspicion of AIBD in comparison with the conventional multistep diagnostic algorithm...
December 28, 2016: Journal of the American Academy of Dermatology
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/28012821/validation-of-chemiluminescent-enzyme-immunoassay-in-detection-of-autoantibodies-in-pemphigus-and-pemphigoid
#12
Yumi Fujio, Kazuo Kojima, Masahiro Hashiguchi, Masatoshi Wakui, Mitsuru Murata, Masayuki Amagai, Jun Yamagami
BACKGROUND: A novel chemiluminescent enzyme immunoassay (CLEIA) was recently developed to quantify autoantibodies specific for desmogleins (Dsgs) and BP180, the target antigens of pemphigus and pemphigoid. This assay is automated and highly accurate and efficient. OBJECTIVE: To validate the use of the CLEIA for detection of autoantibodies during the clinical courses of patients with pemphigus and pemphigoid. METHODS: To define cut-off values for Dsg1, Dsg3, and BP180, we evaluated 47 serum samples from patients with pemphigus foliaceus (PF), 59 from those with pemphigus vulgaris (PV), 52 from those with bullous pemphigoid (BP), and 995 from healthy individuals...
December 6, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/27958610/overexpression-of-psoriasin-s100a7-contributes-to-dysregulated-differentiation-in-psoriasis
#13
Anna-Karin Ekman, Jenny Vegfors, Cecilia Bivik Eding, Charlotta Enerbäck
Psoriasin, which is highly expressed in psoriasis, is encoded by a gene located within the epidermal differentiation complex. The aim of this study was to investigate the effect of endogenous psoriasin on disturbed keratinocyte differentiation in psoriasis. Immunohistochemical staining revealed a gradient of psoriasin expression in the psoriatic epidermis with highest expression in the suprabasal, differentiated layers. Induction of keratinocyte differentiation caused concurrent expression of psoriasin and the differentiation marker involucrin...
December 13, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27881029/pemphigus-herpetiformis-type-drug-reaction-caused-by-erdosteine-containing-mucolytic-in-a-child
#14
Gulsen Akoglu, Sibel Orhun Yavuz
Drug-related pemphigus is very rare in children. Erdosteine is a thiol compound having mucoactive, antioxidant, anti-inflammatory, and antitussive effects and is reported to be safe for treatment of acute respiratory tract diseases in children. Herein, we report a 9-year-old boy presented with pemphigus herpetiformis associated with anti-desmoglein 1 antibodies due to erdosteine consumption.
November 23, 2016: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/27867744/iga-pemphigus-showing-iga-antibodies-to-desmoglein-1-and-3
#15
Salama Hegazy, Sana Bouchouicha, Aida Khaled, Lilia Laadher, Maryem Kallel Sellami, Faten Zeglaoui
BACKGROUND: IgA pemphigus is a rare autoimmune vesiculo-pustular skin disease. Only approximately 70 cases have been reported to date. We report a case of IgA pemphigus with IgA antibodies to desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). CASE REPORT: We report the case of an 60-year-old man with intraepidermal neutrophilic IgA pemphigus with IgA antibodies to Dsg1 and Dsg3. Histologic examination revealed subcorneal neutrophilic pustules with few acantholytic cells...
October 2016: Dermatology Practical & Conceptual
https://www.readbyqxmd.com/read/27792691/-hla-class-ii-in-mexican-patients-with-pemphigus-vulgaris-shared-epitope-for-autoimmunity
#16
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/27765722/staphylococcus-aureus-induces-increased-serine-protease-activity-in-keratinocytes
#17
Michael R Williams, Teruaki Nakatsuji, James A Sanford, Alison F Vrbanac, Richard L Gallo
Bacteria that reside on the skin can influence the behavior of the cutaneous immune system, but the mechanisms responsible for these effects are incompletely understood. Colonization of the skin by Staphylococcus aureus (S. aureus) is increased in atopic dermatitis and can result in increased severity of the disease. In this study, we show that S. aureus stimulates human keratinocytes to increase their endogenous protease activity, including specific increases in trypsin activity. This increased protease activity coincided with increased expression of mRNA for kallikreins (KLKs), with KLK6, 13, and 14 showing the greatest induction after exposure to S...
February 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27750289/pemphigus-autoantibodies-induce-blistering-in-human-conjunctiva
#18
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/27703000/palmitoylation-of-desmoglein-2-is-a-regulator-of-assembly-dynamics-and-protein-turnover
#19
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/27681076/novel-biomarkers-that-assist-in-accurate-discrimination-of-squamous-cell-carcinoma-from-adenocarcinoma-of-the-lung
#20
Kazuya Takamochi, Hiroko Ohmiya, Masayoshi Itoh, Kaoru Mogushi, Tsuyoshi Saito, Kieko Hara, Keiko Mitani, Yasushi Kogo, Yasunari Yamanaka, Jun Kawai, Yoshihide Hayashizaki, Shiaki Oh, Kenji Suzuki, Hideya Kawaji
BACKGROUND: Targeted therapies based on the molecular and histological features of cancer types are becoming standard practice. The most effective regimen in lung cancers is different between squamous cell carcinoma (SCC) and adenocarcinoma (AD). Therefore a precise diagnosis is crucial, but this has been difficult, particularly for poorly differentiated SCC (PDSCC) and AD without a lepidic growth component (non-lepidic AD). Biomarkers enabling a precise diagnosis are therefore urgently needed...
September 29, 2016: BMC Cancer
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