is responsible for a wide variety of cutaneous infections ranging from superficial impetigo to fulminant invasive necrotizing fasciitis. Dysfunction of desmosomes is associated with the pathogenesis of cutaneous diseases. We identified streptococcal pyrogenic exotoxin B (SpeB) as a proteolytic factor that cleaves the extracellular domains of desmoglein 1 and 3. In an epicutaneous infection model, lesional skin infected with an deletion mutant were significantly smaller as compared to those caused by the wild-type strain. Furthermore, immunohistological analysis indicated cleavage of desmogleins that developed around the invasion site of the wild-type strain. In contrast, the mutant was preferentially found on the epidermis surface layer. Taken together, our findings provide evidence that SpeB-mediated degradation of desmosomes has a pathogenic role in development of cutaneous infection.
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http://dx.doi.org/10.3389/fcimb.2018.00010 | DOI Listing |
Acta Paediatr
February 2025
Department of Paediatrics, School of Medicine, University of Crete, Heraklion, Greece.
Aim: The aim of this review was to summarise the current knowledge on host-related factors that contribute to the development and severity of staphylococcal scalded skin syndrome (SSSS) in children.
Methods: A comprehensive assessment and analysis of the existing literature on SSSS clinical features, pathogenesis and susceptibility factors.
Results: SSSS is a blistering skin disease caused by circulating exfoliative toxins (ETs) of Staphylococcus aureus (S.
Cell Mol Life Sci
July 2023
Chair of Vegetative Anatomy, Faculty of Medicine, Institute of Anatomy, Ludwig-Maximilians-University (LMU) Munich, Pettenkoferstrasse 11, 80336, Munich, Germany.
Aims: Arrhythmogenic cardiomyopathy (AC) is a severe heart disease predisposing to ventricular arrhythmias and sudden cardiac death caused by mutations affecting intercalated disc (ICD) proteins and aggravated by physical exercise. Recently, autoantibodies targeting ICD proteins, including the desmosomal cadherin desmoglein 2 (DSG2), were reported in AC patients and were considered relevant for disease development and progression, particularly in patients without underlying pathogenic mutations. However, it is unclear at present whether these autoantibodies are pathogenic and by which mechanisms show specificity for DSG2 and thus can be used as a diagnostic tool.
View Article and Find Full Text PDFFront Cell Dev Biol
January 2023
Chair of Vegetative Anatomy, Institute of Anatomy, Faculty of Medicine, Ludwig-Maximilian-University (LMU), Munich, Germany.
A Disintegrin And Metalloprotease (ADAM) family proteins are involved in several cardiac diseases, and some ADAMs have been associated with cardiomyopathies. ADAM17 is known to cleave desmoglein 2 (DSG2), one of the proteins involved in the pathogenesis of arrhythmogenic cardiomyopathy (AC). Desmosomal stability is impaired in AC, an inheritable genetic disease, the underlying causes of which can be mutations in genes coding for proteins of the desmosome, such as DSG2, desmoplakin (DP), plakoglobin (PG), plakophilin 2 or desmocollin 2.
View Article and Find Full Text PDFBr J Dermatol
January 2023
Department of Dermatology, Peking University First Hospital, Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, National Clinical Research Center for Skin and Immune Diseases, Beijing 100034, China.
Background: Mendelian disorders of cornification (MeDOC) are a group of heterogeneous genodermatoses with different genetic bases. The pathogenesis of a substantial group of MeDOC remains to be elucidated.
Objectives: To identify a new causative gene and the pathogenesis of a previously undescribed autosomal-dominant cornification disorder.
Medicina (Kaunas)
October 2021
Department of Dermatology, University of Florida College of Medicine, Gainesville, FL 32610, USA.
Staphylococcal scalded skin syndrome (SSSS) and bullous impetigo are infections caused by . The pathogenesis of both conditions centers around exotoxin mediated cleavage of desmoglein-1, which results in intraepidermal desquamation. Bullous impetigo is due to the local release of these toxins and thus, often presents with localized skin findings, whereas SSSS is from the systemic spread of these toxins, resulting in a more generalized rash and severe presentation.
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