2002
DOI: 10.1046/j.1523-1747.2002.01720.x
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Abstract: Bullous pemphigoid is a subepidermal autoimmune blistering disease associated with autoantibodies to the hemidesmosomal bullous pemphigoid antigens 180 and 230. Most sera from bullous pemphigoid patients recognize epitopes within the N-terminal NC16A portion of the bullous pemphigoid 180 ectodomain. Using cryosections of human skin, patients' sera were shown to generate dermal-epidermal separation when coincubated with leukocytes and complement from healthy volunteers; however, the specificity of pathogenic au… Show more

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Cited by 170 publications
(208 citation statements)
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“…In experimental BP, subepidermal blistering triggered by rabbit antimBP180 IgG depends on neutrophils and neutrophil-released proteolytic enzymes (18,54,55). In human BP, several lines of evidence using in vitro skin culture and cryosections systems demonstrated that dermal-epidermal separation induced by anti-BP180 autoantibodies also depends on neutrophils (58,59). However, another study showed that anti-BP180 autoantibodies are directly pathogenic without the need of neutrophils in the human skin culture system and SCID mice grafted with human skin (60).…”
Section: Discussionmentioning
confidence: 99%
“…In experimental BP, subepidermal blistering triggered by rabbit antimBP180 IgG depends on neutrophils and neutrophil-released proteolytic enzymes (18,54,55). In human BP, several lines of evidence using in vitro skin culture and cryosections systems demonstrated that dermal-epidermal separation induced by anti-BP180 autoantibodies also depends on neutrophils (58,59). However, another study showed that anti-BP180 autoantibodies are directly pathogenic without the need of neutrophils in the human skin culture system and SCID mice grafted with human skin (60).…”
Section: Discussionmentioning
confidence: 99%
“…The staining intensity of immunoreactants in the skin of immunized mice was assessed semiquantitatively using a score comprising 0, for no staining; 1, faint staining; 2, medium; and 3, intense staining. Detection of serum autoantibodies in mice followed published protocols with minor modifications (8,28). Briefly, after incubating with diluted mouse serum, the frozen sections of murine and human skin were treated with 100-fold diluted Abs to mouse IgG and IgG subclasses.…”
Section: If Microscopy and Immunoblot Analysismentioning
confidence: 99%
“…Immunoadsorption of mouse serum with cell lysate of bacteria transformed with wild-type pGEX was performed, as described (8). Rabbit Abs SA8009 and SA2954 generated against rGST fusion proteins containing sequences of human type XVII collagen (GST-NC16A2-4) and murine type VII collagen (GSTmCOL7C), respectively, were described previously (8,28).…”
Section: If Microscopy and Immunoblot Analysismentioning
confidence: 99%
“…In this model, neutrophils line up along the BMZ and cause detachment of the dermal-epidermal junction through the release of proteolytic enzymes [20]. Mouse models deficient in neutrophils do not form blisters, and similar resistance is seen when neutrophil infiltration is blocked [47].…”
Section: Inflammatory Cell Actionmentioning
confidence: 99%
“…The positive correlation between the titer of IgG (predominately IgG1 and IgG4) to extracellular epitopes of BP180 and BP disease severity is well recognized. In vitro studies and animal models have demonstrated the critical role of Fc receptor-mediated effects in blister formation [19][20][21]. Liu et al described an animal model in which purified rabbit anti-murine BP180 was passively transferred into neonatal mice.…”
Section: Humoral Immune Responsementioning
confidence: 99%