Corrigendum: Cutaneous kinase activity correlates with treatment outcomes following PI3K delta inhibition in mice with experimental pemphigoid diseases

Ghorbanalipoor, Saeedeh; Emtenani, Shirin; Parker, Melissa; Kamaguchi, Mayumi; Osterloh, Colin; Pigors, Manuela; Gross, Natalie; Khil’chenko, Stanislav; Kasprick, Anika; Patzelt, Sabrina; Wortmann, Diana; Ibrahim, Ibrahim O.; Izumi, Kiyotaka; Goletz, Stephanie; Boch, Katharina; Kalies, Kathrin; Bieber, Katja; Smith, Pete; Schmidt, Enno; Ludwig, Ralf J.

doi:10.3389/fimmu.2022.1099535

Cited by 3 publications

(1 citation statement)

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“…In line with previous findings, methylprednisolone as another first-line therapy for MMP, was also able to reduce the severity of skin, although not oral lesions in this mouse model. In this study, Ghorbanalipoor et al also showed that parsaclisib, a selective inhibitor of phosphoinositide 3-kinase delta (PI3Kd) significantly reduced skin and oral mucosal lesions (99,100). With regard to the characteristic symptom of scarring, typically occurring at the eyes of anti-laminin 332 MMP patients, this mouse model may also be suitable to unravel signaling pathways that contributes to this specific immunopathogenesis.…”

Section: Pathophysiology Of Anti-laminin 332 Pemphigoidmentioning

confidence: 65%

Autoimmunity against laminin 332

Patzelt,

Schmidt

2023

Front. Immunol.

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Laminin 332 is a heterotrimeric structural protein of the basal membrane zone (BMZ) of the skin and adjacent mucosal tissues. The importance of laminin 332 for the structural integrity of the BMZ is demonstrated by mutations in any of the three genes encoding for its three chains causing variants of junctional epidermolysis bullosa. Autoimmunity against laminin 332 is observed in mucous membrane pemphigoid (MMP) and in the rare patients with orf-induced pemphigoid. MMP is an autoimmune blistering disease with predominant mucosal manifestations and autoantibodies against the BMZ of the skin and orifice-close mucous membranes. The main autoantigens of MMP are type XVII collagen (BP180) and laminin 332 targeted in about 80% and 10-20% of patients, respectively. An increasing number of studies has highlighted the association of anti-laminin 332 MMP and malignancies that can be revealed in about a quarter of these patients. This data has led to the recommendation of current guidelines to assay for anti-laminin 332 reactivity in all MMP patients. The present review focuses on anti-laminin 332 MMP describing clinical features, its pathophysiology, and detection of serum anti-laminin 332 IgG. In addition, the available data about the occurrence of malignancies in anti-laminin 332 MMP, the underlying tumor entities, and its biology are detailed.

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Section: Pathophysiology Of Anti-laminin 332 Pemphigoidmentioning

confidence: 65%

Autoimmunity against laminin 332

Patzelt,

Schmidt

2023

Front. Immunol.

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Immunpathogenese des Schleimhautpemphigoids

Schmidt

Patzelt

2023

Ophthalmologie

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Granzyme B inhibition reduces autoantibody‐induced dermal–epidermal separation in an ex vivo model of epidermolysis bullosa acquisita

Emtenani,

Lebhar,

Marquis

et al. 2024

Experimental Dermatology

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The pemphigoid disease epidermolysis bullosa acquisita (EBA) is an autoimmune blistering skin disease characterized by autoantibodies against type VII collagen (COL7), immune cell infiltrates at the dermal–epidermal junction and subepidermal blistering. Proteases, particularly granzyme B (GzmB), have been established as therapeutic targets for the treatment of EBA and other pemphigoid diseases. We investigated the impact of the novel GzmB inhibitor SNT‐6935 on anti‐COL7 IgG‐induced subepidermal blistering in a well‐established EBA ex vivo model. Our findings demonstrate that pharmacological targeting of GzmB with its selective inhibitor SNT‐6935 significantly reduced autoantibody‐induced dermal–epidermal separation in human skin cryosections. Interestingly, treatment of skin cryosections with recombinant human GzmB alone did not cause dermal–epidermal separation, suggesting that additional mechanisms alongside GzmB are required for tissue damage in EBA. In conclusion, our study highlights the significant contribution of GzmB to the pathogenesis of EBA and supports the notion of GzmB as a therapeutic target in EBA and other pemphigoid diseases.

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Corrigendum: Cutaneous kinase activity correlates with treatment outcomes following PI3K delta inhibition in mice with experimental pemphigoid diseases

Abstract: A Corrigendum onCutaneous kinase activity correlates with treatment outcomes following PI3K delta inhibition in mice with experimental pemphigoid diseases.

Cited by 3 publications

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Autoimmunity against laminin 332

Autoimmunity against laminin 332

Immunpathogenese des Schleimhautpemphigoids

Granzyme B inhibition reduces autoantibody‐induced dermal–epidermal separation in an ex vivo model of epidermolysis bullosa acquisita

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