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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease hartnup disease
Comorbidity C0030805|bullous pemphigoid
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PubMedID- 21619684 The pathogenic relevance of igg autoantibodies against bp180 is supported by several lines of evidence: 1) the transplacental transfer of pemphigoid igg autoantibodies from mothers to the fetus induces transient skin blistering in the newborn [14-16]; 2) serum levels of igg autoantibodies against bp180 correlate with disease activity in patients with bullous pemphigoid and pemphigoid gestationis [17-20]; 3) patients autoantibodies against bp180 recruit leukocytes to the dermal-epidermal junction and induce dermal-epidermal separation of human skin [21,22]; 4) igg antibodies against bp180 induce subepidermal blistering when passively transferred into neonatal autoantigen humanized, wild type mice and hamsters [23-26]; 5) grafting of human bp180 transgenic mouse skin induces an autoimmune response resulting in subepidermal blistering in wild-type animals [27,28].

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