title: Autoantibodies of non-inflammatory bullous pemphigoid hardly deplete type XVII collagen of keratinocytes.
abstract: Type XVII collagen (COL17) and the non-collagenous 16A (NC16A) domain is regarded as the major pathogenic domains for bullous pemphigoid (BP). Some patients with BP have autoantibodies against parts of COL17 outside the NC16A domain (hereinafter the non-NC16A domain) and show less inflammatory manifestations. There were no significant differences in titres and IgG subclasses between NC16A-BP and non-NC16A-BP as determined by indirect immunofluorescent microscopy. The neutrophil activation capacities determined by ROS release did not differ between NC16A-BP and non-NC16A-BP. However, NC16A-BP IgG depleted COL17 in a dose-dependent manner. Treatment with NC16A-BP IgG, but not with non-NC16A-BP IgG, significantly decreased the adhesion strength. We speculate that the differences in clinical severity between NC16A-BP and non-NC16A-BP relate to the degree of COL17 depletion.
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Imafuku
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Hiroaki
authors.identifier: 0000-0002-8781-2735
authors.initials: H
authors.lastname: Iwata
authors.affiliation: Department of Oral Diagnosis and Medicine, Hokkaido University, Graduate School of Dental Medicine, Sapporo, Japan.
authors.forename: Mayumi
authors.initials: M
authors.lastname: Kamaguchi
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Izumi
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Ken
authors.initials: K
authors.lastname: Natsuga
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Ujiie
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Wataru
authors.initials: W
authors.lastname: Nishie
authors.affiliation: Department of Dermatology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000502:physiology*; D001324:Autoantigens / Q000276:immunology* / Q000378:metabolism; D002478:Cells, Cultured; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000502:physiology; D015603:Keratinocytes / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000378:metabolism; D024041:Non-Fibrillar Collagens / Q000276:immunology* / Q000378:metabolism; D009924:Organ Culture Techniques; D010391:Pemphigoid, Bullous / Q000276:immunology*; D017382:Reactive Oxygen Species / Q000378:metabolism; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D007074:Immunoglobulin G; D024041:Non-Fibrillar Collagens; D017382:Reactive Oxygen Species; D000097805:Collagen Type XVII
keywords: bullous pemphigoid; depletion; internalization; non-collagenous 16A domain; type XVII collagen