2022
DOI: 10.3390/antib11030044
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Deciphering the Contribution of BP230 Autoantibodies in Bullous Pemphigoid

Abstract: Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease predominantly affecting elderly patients and carries significant morbidity and mortality. Patients typically suffer from severe itch with eczematous lesions, urticarial plaques, and/or tense blisters. BP is characterized by the presence of circulating autoantibodies against two components of the hemidesmosome, BP180 and BP230. The transmembrane BP180, also known as type XVII collagen or BPAG2, represents the primary pathogenic autoantigen … Show more

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Cited by 7 publications
(3 citation statements)
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“…Over 90% of BP patients have IgG autoantibodies that target the extracellular membrane-proximal portion (NC16A domain) of BP180. However, circulating anti-BP230 IgG and IgE autoantibodies are seen in up to 80% and 68%, respectively [ 26 ].…”
Section: Discussionmentioning
confidence: 99%
“…Over 90% of BP patients have IgG autoantibodies that target the extracellular membrane-proximal portion (NC16A domain) of BP180. However, circulating anti-BP230 IgG and IgE autoantibodies are seen in up to 80% and 68%, respectively [ 26 ].…”
Section: Discussionmentioning
confidence: 99%
“…The presence of circulating and tissue-bound IgG autoantibodies directed against BP180 and BP230 [ 41 ].…”
Section: Figurementioning
confidence: 99%
“…Similar to BP, inflammation is the major trigger of the early phases of the atherosclerotic process and inflammatory cytokines are associated with a higher risk for developing cardiovascular diseases (75). Next to BP-230-a and BP-230-e, a third isoform named BP-230-b is known (76). This isoform is mainly expressed in the myocardium and skeletal muscle of mice (77).…”
Section: Cardiovascular and Metabolic Diseasesmentioning
confidence: 99%