The spectrum of Castleman diseases has expanded over the past three decades. The phenotype of the diseases varies not only among the three major types but also according to the patient ancestry. Summary Under the Castleman disease (CD) eponym, three distinct diseases sharing common pathological features have been described over time.
Eric Oksenhendler +4 more
wiley +1 more source
Pemphigus IgG Causes Skin Splitting in the Presence of Both Desmoglein 1 and Desmoglein 3 [PDF]
According to the desmoglein (Dsg) compensation concept, different epidermal cleavage planes observed in pemphigus vulgaris and pemphigus foliaceus have been proposed to be caused by different autoantibody profiles against the desmosomal proteins Dsg 1 and Dsg 3.
Volker, Spindler +3 more
openaire +2 more sources
BackgroundA novel fully automated chemiluminescent immunoassay (CLIA) has been developed to quantify autoantibodies specific for desmogleins (Dsg1, Dsg3), BP180, and BP230, key target antigens in pemphigus and bullous pemphigoid.
Na Zhang +14 more
doaj +1 more source
The metalloproteinase ADAM17 promotes acute lung inflammatory responses during pancreatitis
Background and Purpose Acute pancreatitis (AP) is a multifactorial upper gastrointestinal inflammatory disorder that in severe cases (~20% of all AP) is associated with substantial morbidity and mortality, the latter coincident with multiorgan dysfunction, particularly acute lung injury (ALI).
Shermin Chan +17 more
wiley +1 more source
The disease mutation A77V in Ryanodine receptor RyR2 induces changes in energy conduction pathways in the protein [PDF]
Energetically responsive residues of the 217 amino acid N-terminal domain of the cardiac Ryanodine receptor RyR2 are identified by a simple elastic net model. These residues lie along a hydrogen bonded path through the protein.
Burak Erman, Nazan Walpoth
core +1 more source
Characterization of a three-dimensional mucosal equivalent: Similarities and differences with native oral mucosa [PDF]
The aim of this study was to create and characterize a tissue-engineered mucosal equivalent (TEM) that closely resembles native mucosa. TEM consists of human primary keratinocytes and fibroblasts isolated from biopsies taken from healthy donors and ...
Hovius, S.E.R. (Steven) +4 more
core +4 more sources
Cyclophosphamide is associated with long‐term treatment‐free remission in patients with pemphigus
Summary Background and objective Pemphigus treatment has seen significant advancements, yet long‐term remission remains a challenge. Comparative data on the long‐term effectiveness of available therapies are limited. We compared 10‐year relapse rates in pemphigus patients treated with azathioprine or cyclophosphamide plus high‐dose IV corticosteroids ...
Bettina Santler +5 more
wiley +1 more source
IgA pemphigus showing IgA antibodies to desmoglein 1 and 3
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).
Salama Hegazy +5 more
doaj +1 more source
Proteomic analysis of skin invasion by blood fluke larvae. [PDF]
BackgroundDuring invasion of human skin by schistosome blood fluke larvae (cercariae), a multicellular organism breaches the epidermis, basement membrane, and dermal barriers of skin.
Braschi, Simon +7 more
core +1 more source
Multigene Next-Generation Sequencing Panel Identifies Pathogenic Variants in Patients with Unknown Subtype of Epidermolysis Bullosa: Subclassification with Prognostic Implications [PDF]
Purpose: Epidermolysis bullosa (EB), the prototype of heritable blistering diseases, is caused by mutations in as many as 19 distinct genes. In this study, we evaluated the molecular basis of EB in 93 families, many of them of unknown subtype. Methods:
Abiri, Maryam +15 more
core +1 more source

