Enhanced NLRP3 and DEFA1B Expression During the Active Stage of Parenchymal Neuro-Behçet's Disease

Uğurel, Elif; Erdağ, Ece; Küçükali, Cem İ̇smail; Olcay, Ayca; Şanlı, Elif; Akbayır, Ece; Kürtüncü, Murat; Gündüz, Tuncay; Yılmaz, Vuslat; Tüzün, Erdem; Vural, Burçak

doi:10.21873/invivo.11629

Cited by 10 publications

(7 citation statements)

References 28 publications

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“…It seems that an increased serum level of α-defensins 1-3 is due to increased α-defensins 1-3 load in primary granules of neutrophils and increased neutrophil degranulation not due to increased number of neutrophils [26]. Elif Ugurel et al [27] revealed that innate immunity is primarily involved in acute attacks of neuro-Behcet's disease (NBD) and treatment regimens attacking neutrophils might prove efficacy in the treatment of NBD and perhaps replace steroids that are already used for this disease.…”

Section: Discussionmentioning

confidence: 99%

The significance of α-defensins 1-3 in Behcet’s disease: a case-control study among Egyptian patients

Shaat

Meadawy

Rizk

et al. 2020

Egypt Rheumatol Rehabil

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Background Behcet’s disease (BD) is associated with uveitis, oral ulcers, genital ulcers and arthritis. Enhanced activity of innate immunity components, such as neutrophils, natural killer (NK) cells and endothelial cells, is a prominent feature of BD. The aim of this study was to clarify the significance of α-defensins 1-3 in BD and detect their correlation with disease activity, severity and oral ulcer activity. Results Significant differences were found between Behcet’s patients and controls as regards serum α-defensins 1-3 (median 10.1, IQR 4–45 μg/ml versus median 7.4, IQR 0.6–13.2 μg/ml, p = 0.001) and salivary α-defensins 1-3 (median 17.4, IQR 5.7–44 μg/ml versus median 8.6, IQR 3.3–11 μg/ml, p < 0.001). Receiver operating characteristic (ROC) showed a fair area under curve (AUC) (AUC = 0.743), with sensitivity of 40% and specificity 100% at cutoff value of 17.3 μg/ml for serum α-defensins 1-3, while salivary α-defensins 1-3 showed excellent AUC (AUC = 0.936), with sensitivity of 93.3% and specificity of 86.7% at cutoff value of 9.8 μg/ml. The levels of serum and salivary α-defensins 1-3 > cutoff value were significantly higher in active severe Behcet’s patients with active oral ulcers (p < 0.001). Conclusion α-Defensins 1-3 may be involved in the pathogenesis of BD and could be valuable markers in the determination of disease activity, severity and oral ulcer activity.

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Section: Discussionmentioning

confidence: 99%

The significance of α-defensins 1-3 in Behcet’s disease: a case-control study among Egyptian patients

Shaat

Meadawy

Rizk

et al. 2020

Egypt Rheumatol Rehabil

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“…These results suggest that IL-32 and NLRP3 inflammasome could be considered as potential biomarkers of vessel inflammation in NBD. Ugurel et al reported that during NBD attacks, the most remarkably up-regulated genes were defensin alpha 1B and NLRP3 [52]. Thus, NLRP3 can be used as a biomarker for estimation of disease activity in NBD.…”

Section: Key Pointsmentioning

confidence: 99%

Vasculitis and the NLRP3 inflammasome

Hamzaoui

2023

Current Opinion in Rheumatology

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Purpose of review Vasculitis are a group of heterogeneous conditions characterized by chronic inflammation of blood vessels, leading to tissue destruction and organ failure. Vasculitis is an inflammatory process in which immune effector cells infiltrate blood vessels and surrounding tissues. The involvement of inflammasomes seems to occur during inflammatory processes. Recent findings Studies have emphasized that genetic susceptibility is an important aspect of the pathogenesis of vasculitis. The innate immune system is a major contributor to these inflammatory diseases, suggesting that the NOD-like receptor family pyrin domain containing 3 (NLRP3) inflammasome plays a key role. NLRP3 activation causes the assembly of a large multiprotein and leads to the secretion of bioactive interleukin (IL)-1β and IL-18 as well as the induction of inflammatory cell death, termed pyroptosis. Accumulating evidence confirms the involvement of this cascade in sterile inflammatory diseases and other vascular diseases. Summary In this review, we will summarize the current state of knowledge regarding the role of NLRP3 inflammasome in vascular diseases, and discuss the potential of the NLRP3 inflammasome as a therapeutic target.

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“…NLRP12−/− mice developed exacerbated uveitis, which was not attributed to inherent T cell dysfunction but depended on enhanced neutrophil and macrophage accumulation, indicating that NLRP12 exerts an anti-inflammatory role to suppress uveitis [ 202 ]. In peripheral blood mononuclear cell samples of patients with Behçet’s disease, which is one of the uveitis-related chronic inflammatory diseases, NLRP3 expression level is upregulated during the onset of disease [ 203 ]. Genetic variants of NLRP3 and NLRP1 gene were found in patients suffering Behçet’s disease.…”

Section: Pyroptosis In Ocular Diseasesmentioning

confidence: 99%

Spotlight on pyroptosis: role in pathogenesis and therapeutic potential of ocular diseases

Chen

Rong

Xia

2022

J Neuroinflammation

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Pyroptosis is a programmed cell death characterized by swift plasma membrane disruption and subsequent release of cellular contents and pro-inflammatory mediators (cytokines), including IL‐1β and IL‐18. It differs from other types of programmed cell death such as apoptosis, autophagy, necroptosis, ferroptosis, and NETosis in terms of its morphology and mechanism. As a recently discovered form of cell death, pyroptosis has been demonstrated to be involved in the progression of multiple diseases. Recent studies have also suggested that pyroptosis is linked to various ocular diseases. In this review, we systematically summarized and discussed recent scientific discoveries of the involvement of pyroptosis in common ocular diseases, including diabetic retinopathy, age-related macular degeneration, AIDS-related human cytomegalovirus retinitis, glaucoma, dry eye disease, keratitis, uveitis, and cataract. We also organized new and emerging evidence suggesting that pyroptosis signaling pathways may be potential therapeutic targets in ocular diseases, hoping to provide a summary of overall intervention strategies and relevant multi-dimensional evaluations for various ocular diseases, as well as offer valuable ideas for further research and development from the perspective of pyroptosis.

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Enhanced NLRP3 and DEFA1B Expression During the Active Stage of Parenchymal Neuro-Behçet's Disease

Cited by 10 publications

References 28 publications

The significance of α-defensins 1-3 in Behcet’s disease: a case-control study among Egyptian patients

The significance of α-defensins 1-3 in Behcet’s disease: a case-control study among Egyptian patients

Vasculitis and the NLRP3 inflammasome

Spotlight on pyroptosis: role in pathogenesis and therapeutic potential of ocular diseases

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