title: IgG reactivity against non-conformational NH-terminal epitopes of the desmoglein 3 ectodomain relates to clinical activity and phenotype of pemphigus vulgaris.
abstract: Pemphigus vulgaris (PV) is an autoimmune disease caused by immunoglobulin G (IgG) autoantibodies against the desmosomal adhesion molecules, desmoglein (Dsg)3 and Dsg1. The aim of the study was to relate IgG reactivity of 123 PV sera and 40 control sera against NH(2)-terminal non-conformational epitopes of Dsg3 and Dsg1 with disease activity and clinical phenotype by enzyme-linked immunosorbent assay. The results show that (i) the overall reactivity and the titres of IgG reactive with the Dsg3 ectodomain, Dsg3(1-566), significantly correlated with the disease activity of the PV patients; (ii) IgG reactivity against the NH(2)-terminus of Dsg3, Dsg3(1-161), was associated with active PV while there was no direct correlation between the IgG titres and the disease activity; (iii) IgG reactivity against the NH(2)-terminus of Dsg3, Dsg3(1-161), was associated with mucosal and mucocutaneous PV; (iv) IgG titres against a small stretch of the NH(2)-terminus of Dsg3, Dsg3(25-88), were associated with active PV; and (v) IgG in the PV sera detected non-conformational epitopes in addition to the previously identified conformation-dependent epitopes of the Dsg3 and Dsg1 ectodomains.
authors.affiliation: Department of Dermatology, University of Marburg, Marburg, Germany. ralf.mueller@med.uni-marburg.de
authors.forename: Ralf
authors.initials: R
authors.lastname: Müller
authors.forename: Vera
authors.initials: V
authors.lastname: Svoboda
authors.forename: Elke
authors.initials: E
authors.lastname: Wenzel
authors.forename: Susanne
authors.initials: S
authors.lastname: Gebert
authors.forename: Nicolas
authors.initials: N
authors.lastname: Hunzelmann
authors.forename: Hans-Helge
authors.initials: HH
authors.lastname: Müller
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
mesh_terms: D016367:Baculoviridae / Q000378:metabolism; D003001:Cloning, Molecular; D051184:Desmoglein 3 / Q000737:chemistry*; D004797:Enzyme-Linked Immunosorbent Assay; D000939:Epitopes; D005822:Genetic Vectors; D006801:Humans; D007074:Immunoglobulin G / Q000737:chemistry*; D009092:Mucous Membrane / Q000378:metabolism; D010392:Pemphigus / Q000276:immunology*; D010641:Phenotype; D011379:Prognosis; D011487:Protein Conformation; D017434:Protein Structure, Tertiary; D011994:Recombinant Proteins / Q000737:chemistry
chemical_list: D051184:Desmoglein 3; D000939:Epitopes; D007074:Immunoglobulin G; D011994:Recombinant Proteins