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https://www.readbyqxmd.com/read/29775428/the-involvement-of-human-monogenic-cardiomyopathy-genes-in-experimental-polygenic-cardiac-hypertrophy
#1
Priscilla R Prestes, Francine Z Marques, Guillermo Lopez-Campos, Paul Lewandowski, Lea M D Delbridge, Fadi J Charchar, Stephen B Harrap
Hypertrophic cardiomyopathy thickens heart muscles reducing functionality and increasing risk of cardiac disease and morbidity. Genetic factors are involved, but their contribution is poorly understood. We used the hypertrophic heart rat (HHR), a unique normotensive polygenic model of cardiac hypertrophy and heart failure to investigate the role of genes associated with monogenic human cardiomyopathy. We selected 42 genes involved in monogenic human cardiomyopathies to study: 1) DNA variants, by sequencing the whole-genome of 13-week old HHR and age-matched normal heart rat (NHR), its genetic control strain; 2) mRNA expression, by targeted RNA-sequencing in left ventricles of HHR and NHR at five ages (2-days old, 4-, 13-, 33- and 50-weeks old) compared to human idiopathic dilated data; and 3) microRNA expression, with rat microRNA microarrays in left ventricles of 2-days old HHR and age-matched NHR...
May 18, 2018: Physiological Genomics
https://www.readbyqxmd.com/read/29770335/the-significance-of-scalp-involvement-in-pemphigus-a-literature-review
#2
REVIEW
Marta Sar-Pomian, Lidia Rudnicka, Malgorzata Olszewska
Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29755451/autoantibody-signaling-in-pemphigus-vulgaris-development-of-an-integrated-model
#3
REVIEW
Thomas Sajda, Animesh A Sinha
Pemphigus vulgaris (PV) is an autoimmune skin blistering disease effecting both cutaneous and mucosal epithelia. Blister formation in PV is known to result from the binding of autoantibodies (autoAbs) to keratinocyte antigens. The primary antigenic targets of pathogenic autoAbs are known to be desmoglein 3, and to a lesser extent, desmoglein 1, cadherin family proteins that partially comprise the desmosome, a protein structure responsible for maintaining cell adhesion, although additional autoAbs, whose role in blister formation is still unclear, are also known to be present in PV patients...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29753032/enhancement-of-cutaneous-wound-healing-by-dsg2-augmentation-of-upar-secretion
#4
Felicia Cooper, Andrew M Overmiller, Anthony Loder, Donna M Brennan-Crispi, Kathleen P McGuinn, Molly R Marous, Theresa A Freeman, Natalia A Riobo-Del Galdo, Linda D Siracusa, James K Wahl, Mỹ G Mahoney
In addition to playing a role in adhesion, desmoglein 2 (Dsg2) is an important regulator of growth and survival signaling pathways, cell proliferation, migration and invasion, and oncogenesis. While low-level Dsg2 expression is observed in basal keratinocytes and is downregulated in non-healing venous ulcers, overexpression has been observed in both melanomas and non-melanoma malignancies. Here, we show that transgenic mice overexpressing Dsg2 in basal keratinocytes primed the activation of mitogenic pathways, but did not induce dramatic epidermal changes or susceptibility to chemical-induced tumor development...
May 9, 2018: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/29749496/increased-expression-of-microrna-338-3p-contributes-to-production-of-dsg3-antibody-in-pemphigus-vulgaris-patients
#5
Qingxiu Liu, Feiyi Cui, Menglei Wang, Hao Xiong, Xiaoming Peng, Liuping Liang, Li Li, Jing Zhang, Xuebiao Peng, Kang Zeng
Expression of microRNA-338-3p (miR-338-3p) was aberrantly elevated in pemphigus vulgaris (PV), although its role in PV is still unknown. The present study investigated the functional role and possible molecular mechanisms of miR-338-3p in PV. Reverse transcription-quantitative polymerase chain reaction (RT-qPCR) was performed to detect miR-338-3p expression in peripheral blood mononuclear cells (PBMCs) from patients with PV. Correlation with disease severity and anti-desmoglein 3 antibody (anti-Dsg3) titers were analyzed in patients with PV...
April 26, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29740444/keratinocyte-binding-assay-identifies-anti-desmosomal-pemphigus-antibodies-where-other-tests-are-negative
#6
Federica Giurdanella, Albertine M Nijenhuis, Gilles F H Diercks, Marcel F Jonkman, Hendri H Pas
The serological diagnosis of pemphigus relies on the detection of IgG autoantibodies directed against the epithelial cell surface by indirect immunofluorescence (IIF) on monkey esophagus and against desmoglein 1 (Dsg1) and Dsg3 by ELISA. Although being highly sensitive and specific tools, discrepancies can occur. It is not uncommon that sera testing positive by ELISA give a negative result by IIF and vice versa . This brings diagnostic challenges wherein pemphigus has to be ascertained or ruled out, especially when no biopsy is available...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29723379/oxidative-stress-in-patients-with-endemic-pemphigus-foliaceus-and-healthy-subjects-with-anti-desmoglein-1-antibodies
#7
Ericson Leonardo Gutierrez, Willy Ramos, Lucia Seminario-Vidal, Mercedes Tello, Gerardo Ronceros, Alex G Ortega-Loayza
BACKGROUND: Previous studies have shown oxidative stress in pemphigus vulgaris and pemphigus foliaceus, nevertheless, it remains unknown whether a similar response is characteristic of endemic pemphigus foliaceus in Peru. OBJECTIVES: To determine the oxidative stress response in endemic pemphigus foliaceus patients and subjects with positive for anti-desmoglein1 antibodies (anti-dsg1) from endemic areas of Peru. SUBJECTS AND METHODS: This is a cross-sectional study...
March 2018: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29705242/epithelial-barrier-dysfunction-in-desmoglein-1-deficiency
#8
Laura Polivka, Smail Hadj-Rabia, Elodie Bal, Stéphanie Leclerc-Mercier, Marine Madrange, Yamina Hamel, Damien Bonnet, Stéphanie Mallet, Hubert Lepidi, Caroline Ovaert, Patrick Barbet, Christophe Dupont, Bénédicte Neven, Arnold Munnich, Lisa M Godsel, Florence Campeotto, Robert Weil, Emmanuel Laplantine, Sylvie Marchetto, Jean-Paul Borg, William I Weis, Jean-Laurent Casanova, Anne Puel, Kathleen J Green, Christine Bodemer, Asma Smahi
Desmoglein-1 deficiency is a missing link between impaired epithelial barrier function and immunological dysregulation. Our characterization of SAMEC syndrome highlighted the pivotal role of the desmoglein-1/ERBIN interaction as an inhibitor of skin inflammation via the NF-κB signaling pathway.
April 26, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29702999/immunological-markers-as-predictors-of-developing-steroid-induced-diabetes-mellitus-in-pemphigus-vulgaris-patients-an-observational-study
#9
Ana Sorina Dănescu, Ioana Bâldea, Daniel Corneliu Leucuţa, Iulia Lupan, Gabriel Samaşca, Cassian Sitaru, Roxana Chiorean, Adrian Baican
The aim of this study was to evaluate the clinical importance of autoantibodies in pemphigus vulgaris patients who developed steroid-induced diabetes mellitus (SID) because of the glucocorticoid therapy of pemphigus.A total of 137 patients with pemphigus vulgaris were studied. Patients with SID and pemphigus were compared with those that had only pemphigus. The variables recorded were: age at diagnosis, sex, body mass index, presence of diabetes mellitus (DM), cumulative cortisone dose, treatment duration, value of anti-desmoglein 1 and 3, and anti-glutamic acid decarboxylase autoantibodies...
April 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29675021/anti-thyroid-peroxidase-reactivity-is-heightened-in-pemphigus-vulgaris-and-is-driven-by-human-leukocyte-antigen-status-and-the-absence-of-desmoglein-reactivity
#10
Kristina Seiffert-Sinha, Shahzaib Khan, Kristopher Attwood, John A Gerlach, Animesh A Sinha
Pemphigus vulgaris (PV) belongs to an autoimmune disease cluster that includes autoimmune thyroid disease (AITD), suggesting common mechanisms driving autoimmune susceptibility. Our group has shown that PV patients exhibit significant reactivity to AITD-related anti-thyroid peroxidase (anti-TPO), and anti-TPO antibodies affect signaling pathways in keratinocytes similar to anti-desmoglein (Dsg) 3 antibodies. To further assess the relevance of anti-TPO reactivity in PV, we analyzed anti-TPO levels in 280 PV and 167 healthy control serum samples across a comprehensive set of variable and static parameters of disease activity and etiopathogenesis...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29643851/atomic-force-microscopy-provides-new-mechanistic-insights-into-the-pathogenesis-of-pemphigus
#11
REVIEW
Franziska Vielmuth, Volker Spindler, Jens Waschke
Autoantibodies binding to the extracellular domains of desmoglein (Dsg) 3 and 1 are critical in the pathogenesis of pemphigus by mechanisms leading to impaired function of desmosomes and blister formation in the epidermis and mucous membranes. Desmosomes are highly organized protein complexes which provide strong intercellular adhesion. Desmosomal cadherins such as Dsgs, proteins of the cadherin superfamily which interact via their extracellular domains in Ca2+ -dependent manner, are the transmembrane adhesion molecules clustered within desmosomes...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29630151/paraneoplastic-pemphigus-without-antibodies-to-desmoglein-1-and-3
#12
Kelsey Flood, Christopher Stroud, Zelmira Lazarov, Nadarajah Vigneswara, Sylvia Hsu
Paraneoplastic pemphigus is a severe autoimmune blistering disease presenting in the setting of underlying malignancy. Paraneoplastic pemphigus is associated with diffuse painful stomatitis throughout the oral cavity with extension to the lips. The cutaneous findings are varied and have been described as lichenoid, pemphigoid, and targetoid lesions. Herein, we report a patient with paraneoplastic pemphigus whose routine testing led to a diagnosis of pemphigus vulgaris. However, further testing was pursued revealing an antibody profile consistent with paraneoplastic pemphigus...
February 15, 2018: Dermatology Online Journal
https://www.readbyqxmd.com/read/29626572/th1-th17-cell-recognition-of-desmoglein-3-and-bullous-pemphigoid-antigen-180-in-lichen-planus
#13
Thomas Schmidt, Farzan Solimani, Robert Pollmann, Ronja Stein, Ansgar Schmidt, Inna Stulberg, Katja Kühn, Rüdiger Eming, Verena Eubel, Peter Kind, Nicole Arweiler, Cassian Sitaru, Michael Hertl
We identified Th1/Th17 cell responses against desmoglein 3 and bullous pemphigoid antigen 180 in lichen planus. In contrast, patients with pemphigus vulgaris and bullous pemphigoid showed significantly higher Th2 cell responses against these autoantigens.
April 4, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29616033/keratins-regulate-p38mapk-dependent-desmoglein-binding-properties-in-pemphigus
#14
Franziska Vielmuth, Elias Walter, Michael Fuchs, Mariya Y Radeva, Fanny Buechau, Thomas M Magin, Volker Spindler, Jens Waschke
Keratins are crucial for the anchorage of desmosomes. Severe alterations of keratin organization and detachment of filaments from the desmosomal plaque occur in the autoimmune dermatoses pemphigus vulgaris and pemphigus foliaceus (PF), which are mainly caused by autoantibodies against desmoglein (Dsg) 1 and 3. Keratin alterations are a structural hallmark in pemphigus pathogenesis and correlate with loss of intercellular adhesion. However, the significance for autoantibody-induced loss of intercellular adhesion is largely unknown...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29609015/comparison-of-ethylenediaminetetraacetic-acid-treated-desmoglein-elisa-and-conventional-desmoglein-elisa-in-the-evaluation-of-pemphigus-vulgaris-in-remission
#15
Maryam Daneshpazhooh, Hamidreza Mahmoudi, Kamran Balighi, Amir Teimourpour, Zohreh Khodashenas, Maryam Ghiasi, Hasan Khosravi, Cheyda Chams-Davatchi
No abstract text is available yet for this article.
March 30, 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29604126/sam-syndrome-is-characterized-by-extensive-phenotypic-heterogeneity
#16
Shahar Taiber, Liat Samuelov, Janan Mohamad, Eran Cohen Barak, Ofer Sarig, Stavit Allon Shalev, Gilles Lestringant, Eli Sprecher
Severe skin dermatitis, multiple allergies and metabolic wasting (SAM) syndrome is a rare life-threatening inherited condition caused by bi-allelic mutations in DSG1 encoding desmoglein 1. The disease was initially reported to manifest with severe erythroderma, failure to thrive, atopic manifestations, recurrent infections, hypotrichosis and palmoplantar keratoderma. We present 3 new cases of SAM syndrome in 2 families and review the cases published so far. Whole exome and direct sequencing were used to identify SAM syndrome-causing mutations...
March 31, 2018: Experimental Dermatology
https://www.readbyqxmd.com/read/29573413/herpetiform-pemphigus-with-characteristic-transmission-electron-microscopic-findings-of-various-sized-ballooning-vacuoles-in-keratinocytes-without-acantholysis
#17
N Ishiura, M Tamura-Nakano, H Okochi, C Tateishi, M Maki, Y Shimoda, N Ishii, T Hashimoto, T Tamaki
We report a unique Japanese female case of herpetiform pemphigus (HP) who had immunoglobulin G (IgG) autoantibodies reactive with non-desmosomal sites of keratinocytes, and presented characteristic transmission electron microscopic (TEM) findings of various-sized vacuoles in keratinocytes without acantholysis. The patient presented with pruritic annular edematous erythemas with small blisters lining at the margins on the trunk and extremities. Histopathological examinations showed intraepidermal blisters with prominent infiltrations of eosinophils...
March 24, 2018: British Journal of Dermatology
https://www.readbyqxmd.com/read/29566126/electrical-dysfunctions-in-human-induced-pluripotent-stem-cell-derived-cardiomyocytes-from-a-patient-with-an-arrhythmogenic-right-ventricular-cardiomyopathy
#18
Ibrahim El-Battrawy, Zhihan Zhao, Huan Lan, Lukas Cyganek, Christoph Tombers, Xin Li, Fanis Buljubasic, Siegfried Lang, Malte Tiburcy, Wolfram-Hubertus Zimmermann, Jochen Utikal, Thomas Wieland, Martin Borggrefe, Xiao-Bo Zhou, Ibrahim Akin
Aims: Our aim is to investigate the arrhythmogenic mechanism in arrhythmogenic right ventricular cardiomyopathy (ARVC)-patients by using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs). Methods and results: Human-induced pluripotent stem cell-derived cardiomyocytes were generated from human skin fibroblasts of two healthy donors and an ARVC-patient with a desmoglein-2 (DSG2) mutation. Patch clamp, quantitative polymerase chain reaction, and calcium imaging techniques were employed for the study...
March 16, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29560011/chronic-paronychia-and-onychomadesis-in-pemphigus-vegetans-an-unusual-presentation-in-a-rare-autoimmune-disease
#19
Thanisorn Sukakul, Supenya Varothai
Pemphigus vegetans is a rare variant of deep acantholytic pemphigus which usually presents with vesiculobullous rash and vegetative plaques on the folds. We report a case of pemphigus vegetans patient who presented with rashes on tips of fingers and toes resembling paronychia and onychomadesis that misled the diagnosis for months. The final diagnosis of Hallopeau-type pemphigus vegetans was made based on histopathology and direct immunofluorescence studies. Interestingly, not only the clinical presentation was atypical, but blood tests for anti-desmoglein 1 and 3 antibodies by ELISA technique were also negative...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29545915/characterization-of-primary-human-mammary-epithelial-cells-isolated-and-propagated-by-conditional-reprogrammed-cell-culture
#20
Liting Jin, Ying Qu, Liliana J Gomez, Stacey Chung, Bingchen Han, Bowen Gao, Yong Yue, Yiping Gong, Xuefeng Liu, Farin Amersi, Catherine Dang, Armando E Giuliano, Xiaojiang Cui
Purpose: Conditional reprogramming methods allow for the inexhaustible in vitro proliferation of primary epithelial cells from human tissue specimens. This methodology has the potential to enhance the utility of primary cell culture as a model for mammary gland research. However, few studies have systematically characterized this method in generating in vitro normal human mammary epithelial cell models. Results: We show that cells derived from fresh normal breast tissues can be propagated and exhibit heterogeneous morphologic features...
February 20, 2018: Oncotarget
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