Pemphigus is a life-threatening autoimmune disease. Several phenotypic variants are part of this family of bullous disorders. The disease is mainly mediated by pathogenic autoantibodies, but is also directed against two desmosomal adhesion proteins, desmoglein 1 (DSG1) and 3 (DSG3), which are expressed in the skin and mucosae. By binding to their antigens, autoantibodies induce the separation of keratinocytes, in a process known as acantholysis. The two main Pemphigus variants are Pemphigus vulgaris and foliaceus. Several models of Pemphigus have been described: in vitro, ex vivo and in vivo, passive or active mouse models. Although no model is ideal, different models display specific characteristics that are useful for testing different hypotheses regarding the initiation of Pemphigus, or to evaluate the efficacy of experimental therapies. Different disease models also allow us to evaluate the pathogenicity of specific Pemphigus autoantibodies, or to investigate the role of previously not described autoantigens. The aim of this review is to provide an overview of Pemphigus disease models, with the main focus being on active models and their potential to reproduce different disease subgroups, based on the involvement of different autoantigens.

In Vitro, Ex Vivo and In Vivo Models for the Study of Pemphigus / Lotti, Roberta; Atene, CLAUDIO GIACINTO; Zanfi Emma, Dorotea; Bertesi, Matteo; Zanocco-Marani, Tommaso. - In: INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES. - ISSN 1422-0067. - 23:13(2022), pp. 7044-7054. [10.3390/ijms23137044]

In Vitro, Ex Vivo and In Vivo Models for the Study of Pemphigus

Lotti Roberta
;
Atene Claudio Giacinto;Bertesi Matteo;Zanocco-Marani Tommaso
2022

Abstract

Pemphigus is a life-threatening autoimmune disease. Several phenotypic variants are part of this family of bullous disorders. The disease is mainly mediated by pathogenic autoantibodies, but is also directed against two desmosomal adhesion proteins, desmoglein 1 (DSG1) and 3 (DSG3), which are expressed in the skin and mucosae. By binding to their antigens, autoantibodies induce the separation of keratinocytes, in a process known as acantholysis. The two main Pemphigus variants are Pemphigus vulgaris and foliaceus. Several models of Pemphigus have been described: in vitro, ex vivo and in vivo, passive or active mouse models. Although no model is ideal, different models display specific characteristics that are useful for testing different hypotheses regarding the initiation of Pemphigus, or to evaluate the efficacy of experimental therapies. Different disease models also allow us to evaluate the pathogenicity of specific Pemphigus autoantibodies, or to investigate the role of previously not described autoantigens. The aim of this review is to provide an overview of Pemphigus disease models, with the main focus being on active models and their potential to reproduce different disease subgroups, based on the involvement of different autoantigens.
24-giu-2022
23
13
7044
7054
In Vitro, Ex Vivo and In Vivo Models for the Study of Pemphigus / Lotti, Roberta; Atene, CLAUDIO GIACINTO; Zanfi Emma, Dorotea; Bertesi, Matteo; Zanocco-Marani, Tommaso. - In: INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES. - ISSN 1422-0067. - 23:13(2022), pp. 7044-7054. [10.3390/ijms23137044]
Lotti, Roberta; Atene, CLAUDIO GIACINTO; Zanfi Emma, Dorotea; Bertesi, Matteo; Zanocco-Marani, Tommaso
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