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Mitochondrial Autoantibodies and the Role of Apoptosis in Pemphigus Vulgaris

by Dana M. Hutchison, Anna-Marie Hosking, Ellen M. Hong and Sergei A. Grando

Antibodies 2022, 11(3), 55; https://doi.org/10.3390/antib11030055 - 25 Aug 2022

Cited by 4 | Viewed by 3346

Pemphigus vulgaris (PV) is an IgG autoantibody-mediated, potentially fatal mucocutaneous disease manifested by progressive non-healing erosions and blisters. Beyond acting to inhibit adhesion molecules, PVIgGs elicit a unique process of programmed cell death and detachment of epidermal keratinocytes termed apoptolysis. Mitochondrial damage by [...] Read more.

Pemphigus vulgaris (PV) is an IgG autoantibody-mediated, potentially fatal mucocutaneous disease manifested by progressive non-healing erosions and blisters. Beyond acting to inhibit adhesion molecules, PVIgGs elicit a unique process of programmed cell death and detachment of epidermal keratinocytes termed apoptolysis. Mitochondrial damage by antimitochondrial antibodies (AMA) has proven to be a critical link in this process. AMA act synergistically with other autoantibodies in the pathogenesis of PV. Importantly, absorption of AMA inhibits the ability of PVIgGs to induce blisters. Pharmacologic agents that protect mitochondrial function offer a new targeted approach to treating this severe immunoblistering disease. Full article

(This article belongs to the Special Issue Advances in the Use of Therapeutic Antibodies and Identification of Novel Autoantibodies in Dermatologic Disease)

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11 pages, 561 KiB

Open AccessEditor’s ChoiceReview

Deciphering the Contribution of BP230 Autoantibodies in Bullous Pemphigoid

by Connor Cole, Luca Borradori and Kyle T. Amber

Antibodies 2022, 11(3), 44; https://doi.org/10.3390/antib11030044 - 28 Jun 2022

Cited by 5 | Viewed by 4407

Abstract

Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease predominantly affecting elderly patients and carries significant morbidity and mortality. Patients typically suffer from severe itch with eczematous lesions, urticarial plaques, and/or tense blisters. BP is characterized by the presence of circulating autoantibodies against [...] Read more.

Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease predominantly affecting elderly patients and carries significant morbidity and mortality. Patients typically suffer from severe itch with eczematous lesions, urticarial plaques, and/or tense blisters. BP is characterized by the presence of circulating autoantibodies against two components of the hemidesmosome, BP180 and BP230. The transmembrane BP180, also known as type XVII collagen or BPAG2, represents the primary pathogenic autoantigen in BP, whereas the intracellular BP230 autoantigen is thought to play a minor role in disease pathogenesis. Although experimental data exist suggesting that anti-BP230 antibodies are secondarily formed following initial tissue damage mediated by antibodies targeting extracellular antigenic regions of BP180, there is emerging evidence that anti-BP230 IgG autoantibodies alone directly contribute to tissue damage. It has been further claimed that a subset of patients has a milder variant of BP driven solely by anti-BP230 autoantibodies. Furthermore, the presence of anti-BP230 autoantibodies might correlate with distinct clinical features. This review summarizes the current understanding of the role of BP230 and anti-BP230 antibodies in BP pathogenesis. Full article

(This article belongs to the Special Issue Advances in the Use of Therapeutic Antibodies and Identification of Novel Autoantibodies in Dermatologic Disease)

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15 pages, 1317 KiB

Open AccessReview

A Review of Acquired Autoimmune Blistering Diseases in Inherited Epidermolysis Bullosa: Implications for the Future of Gene Therapy

by Payal M. Patel, Virginia A. Jones, Christy T. Behnam, Giovanni Di Zenzo and Kyle T. Amber

Antibodies 2021, 10(2), 19; https://doi.org/10.3390/antib10020019 - 17 May 2021

Cited by 7 | Viewed by 4795

Abstract

Gene therapy serves as a promising therapy in the pipeline for treatment of epidermolysis bullosa (EB). However, with great promise, the risk of autoimmunity must be considered. While EB is a group of inherited blistering disorders caused by mutations in various skin proteins, [...] Read more.

Gene therapy serves as a promising therapy in the pipeline for treatment of epidermolysis bullosa (EB). However, with great promise, the risk of autoimmunity must be considered. While EB is a group of inherited blistering disorders caused by mutations in various skin proteins, autoimmune blistering diseases (AIBD) have a similar clinical phenotype and are caused by autoantibodies targeting skin antigens. Often, AIBD and EB have the same protein targeted through antibody or mutation, respectively. Moreover, EB patients are also reported to carry anti-skin antibodies of questionable pathogenicity. It has been speculated that activation of autoimmunity is both a consequence and cause of further skin deterioration in EB due to a state of chronic inflammation. Herein, we review the factors that facilitate the initiation of autoimmune and inflammatory responses to help understand the pathogenesis and therapeutic implications of the overlap between EB and AIBD. These may also help explain whether corrections of highly immunogenic portions of protein through gene therapy confers a greater risk towards developing AIBD. Full article

(This article belongs to the Special Issue Advances in the Use of Therapeutic Antibodies and Identification of Novel Autoantibodies in Dermatologic Disease)

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