Laminin β4 is a constituent of the cutaneous basement membrane zone and additional autoantigen of anti-p200 pemphigoid : = Laminin [Beta]4 is a constituent of the cutaneous basement membrane zone and additional autoantigen of anti-p200 pemphigoid

Abstract: Background
Anti-p200 pemphigoid is a subepidermal autoimmune blistering disease (AIBD) characterized by autoantibodies against a 200 kDa protein. Laminin γ1 has been described as target antigen in 70% to 90% of patients. No diagnostic assay is widely available for anti-p200 pemphigoid, which might be due to the unclear pathogenic relevance of anti-laminin γ1 autoantibodies.

Objective
To identify a target antigen with higher clinical and diagnostic relevance.

Methods
Immunoprecipitation, mass spectrometry, and immunoblotting were employed for analysis of skin extracts and sera of patients with anti-p200 pemphigoid (n = 60), other AIBD (n = 33), and healthy blood donors (n = 29). To localize the new antigen in skin, cultured keratinocytes and fibroblasts, quantitative real-time polymerase chain reaction and immunofluorescence microscopy were performed.

Results
Laminin β4 was identified as target antigen of anti-p200 pemphigoid in all analyzed patients. It was located at the level of the basement membrane zone of the skin with predominant expression in keratinocytes.

Limitations
A higher number of sera needs to be tested to verify that laminin β4 is the diagnostically relevant antigen of anti-p200 pemphigoid.

Conclusion
The identification of laminin β4 as an additional target antigen in anti-p200 pemphigoid will allow its differentiation from other AIBD and as such, improve the management of these rare disorders

Angaben zum Objekt

• Weitere Titel: Laminin [Beta]4 is a constituent of the cutaneous basement membrane zone and additional autoantigen of anti-p200 pemphigoid
• Standort: Deutsche Nationalbibliothek Frankfurt am Main
• Umfang: Online-Ressource
• Sprache: Englisch
• Anmerkungen: Journal of the American Academy of Dermatology. - 90, 4 (2024) , 790-797, ISSN: 1097-6787

Beteiligte, Orts- und Zeitangaben

• Ereignis: Veröffentlichung
• (wo): Freiburg
• (wer): Universität
• (wann): 2023
• Urheber: Goletz, Stephanie ; Pigors, Manuela ; Lari, Tina Rastegar ; Hammers, Christoph M. ; Wang, Yao ; Emtenani, Shirin ; Aumailley, Monique ; Holtsche, Maike M. ; Stang, Felix H. ; Weyers, Imke ; König, Inke R. ; Has, Cristina ; Radzimski, Christiane ; Komorowski, Lars ; Zillikens, Detlef ; Schmidt, Enno

Weitere Informationen

• DOI: 10.1016/j.jaad.2023.11.014
• URN: urn:nbn:de:bsz:25-freidok-2424142
• Rechteinformation: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Letzte Aktualisierung: 25.03.2025, 13:54 MEZ