Pathogenic Epitopes of Autoantibodies in Pemphigus Reside in the Amino-Terminal Adhesive Region of Desmogleins Which Are Unmasked by Proteolytic Processing of Prosequence [PDF]
, 2009 Pemphigus targets desmogleins (Dsgs), which are thought to be synthesized as inactive precursor proteins with prosequences that are cleaved by substilisin-like proprotein convertases, such as furin, to yield mature adhesive molecules.
Ishii, Ken +13 more
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Desmoglein-1, differentiation, and disease [PDF]
Journal of Clinical Investigation, 2013 Desmoglein-1 (DSG1), a desmosomal protein, maintains the structure of epidermis through its adhesive function. However, heterozygous mutations in DSG1 in humans result in abnormal differentiation, as does downregulation of DSG1 in human skin organ culture, suggesting that it may have important signaling functions. In this issue of the JCI, Harmon et al.
Christoph M, Hammers, John R, Stanley
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Case Reports in Oncology, 2021 Pemphigus is a serious and rare chronic bullous autoimmune disease. It is characterized by mucocutaneous erosions secondary to autoantibodies directed against desmogleins 1 and 3, proteins involved in intercellular adhesion mechanisms.
Camille Invernizzi +5 more
doaj +1 more source
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview [PDF]
Anais Brasileiros de Dermatologia, 2019 : Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms.
Celina Wakisaka Maruta +5 more
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Taiwanese dermatological association (TDA) consensus for the management of pemphigus
Journal of the Formosan Medical Association, 2023 Pemphigus is an uncommon but life-threatening autoimmune blistering disease characterized by the presence of antibodies against desmogleins. Without effective treatment, pemphigus can result in significant morbidity and mortality.
Chia-Yu Chu +7 more
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Expression of Desmoglein 1 Compensates for Genetic Loss of Desmoglein 3 in Keratinocyte Adhesion [PDF]
Journal of Investigative Dermatology, 2002 The desmoglein compensation hypothesis, namely that one desmoglein can compensate for loss of function of another, has been proposed to explain the tissue specificity of the autoantibody-induced loss of cell adhesion in pemphigus. To validate this hypothesis genetically, we used desmoglein-3 knockout mice (DSG3-/-) that lose their telogen hair ...
Hanakawa, Yasushi +2 more
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Genetic Evidence for a Novel Human Desmosomal Cadherin, Desmoglein 4 [PDF]
, 2003 Desmosomes are essential adhesion structures in most epithelia that link the intermediate filament network of one cell to its neighbor, thereby forming a strong bond.
Bower, Christopher +3 more
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Correlation of antibodies against desmogleins 1 and 3 with indirect immunofluorescence and disease activity in 72 patients with pemphigus vulgaris [PDF]
, 2017 The enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence (IIF) have both been used for testing of antibodies to desmogleins 1 and 3 (anti-Dsg1 and anti-Dsg3) and for the serologic diagnosis of pemphigus.
Medenica, Ljiljana +3 more
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Autoimmune pemphigus: difficulties in diagnosis and the molecular mechanisms underlying the disease
Frontiers in ImmunologyRecently recognised as a desmosomal disorder, autoimmune pemphigus remains severe in some of its forms, such as pemphigus vulgaris. This review is divided into four parts.
Olga Simionescu, Sorin Ioan Tudorache
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, 2012 (A) Immunofluorescence shows abundant expression of desmogleins (red, panel ii) and desmoplakin (red, panel v) in the WT but not in the Klf5CN corneal epithelium (panels iii and vi, respectively).
Sudha Swamynathan (135203) +4 more
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