Searching for experimental models of Pemphigus vulgaris.
The current knowledge on Pemphigus vulgaris (PV) pathophysiology suggests that blister formation relies on both PV IgG and non-IgG serum factors activity. PV autoimmunity seems to develop against both desmoglein 1/3 and acetylcholine receptors leading to transduction of signals to the cell mediated by phosphorilation events. Serum factors other than IgG also participate to PV acantholysis through apoptotic or cytokine-mediated mechanisms. Apart from the role played by each actor within the acantholysis, however, the current scenario arises important methodological issues. For example, the use of PV IgG or monoclonal anti-Dsg3 antibodies to experimentally reproduce the disease appears inadequate, as it does not take into account the role of non-IgG factors. On the basis of the above observations and those from our laboratories, here we propose that using whole sera from PV patients with active disease represents the most faithful manner to mimic the disease.
Cirillo
Nicola
N
Regional Center on Craniofacial Malformations-MRI, First School of Medicine and Surgery, Second University of Naples, 80138 Naples, Italy. cirillo.sun@libero.it
Gombos
Fernando
F
Ruocco
Vincenzo
V
Lanza
Alessandro
A
D000818:Animals; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001768:Blister / Q000097:blood / Q000276:immunology / Q000503:physiopathology; D051183:Desmoglein 1 / Q000276:immunology; D051184:Desmoglein 3 / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology / Q000502:physiology; D051379:Mice; D008954:Models, Biological; D008962:Models, Theoretical; D010392:Pemphigus / Q000097:blood* / Q000276:immunology* / Q000503:physiopathology; D011950:Receptors, Cholinergic / Q000276:immunology; D044967:Serum / Q000276:immunology
D001323:Autoantibodies; D051183:Desmoglein 1; D051184:Desmoglein 3; D007074:Immunoglobulin G; D011950:Receptors, Cholinergic