Ostadi Moghaddam Amir, Jin Xiaowei, Tajvidi Safa Bahareh, Seiffert-Sinha Kristina, Leiker Merced, Jones Elijah, Zhai Haiwei, Rosenbohm Jordan, Meng Fanben, Sinha Animesh A, Yang Ruiguo
Department of Biomedical Engineering, Michigan State University, East Lansing, MI 48824, USA.
Institute of Quantitative Health Science and Engineering, Michigan State University, East Lansing, MI 48824, USA.
PNAS Nexus. 2025 Jul 18;4(8):pgaf218. doi: 10.1093/pnasnexus/pgaf218. eCollection 2025 Aug.
Pemphigus vulgaris (PV) is a blistering autoimmune disease that affects the skin and mucous membranes. The mechanisms by which PV antibodies induce loss of cohesion in keratinocytes are not fully understood. It is accepted that the process starts with antibody binding to desmosomal targets, which leads to its disassembly and subsequent structural changes to cell-cell adhesions. In vitro imaging of desmosome molecules has been used to characterize this initial phase. However, there remains an untapped potential of image analysis in providing us with more in-depth knowledge regarding biophysical changes after antibody binding. Currently, there is no quantitative framework from immunofluorescence images in PV pathology. Here, we seek to establish a correlation of biophysical changes with antibody pathogenicity by examining the effects of PV antibodies on adhesion molecules and the cytoskeletal network. Specifically, we introduced a data-driven approach to quantitatively evaluate perturbations in adhesion molecules following antibody treatment. We identify distinct imaging signatures that mark the impact of antibody binding on the remodeling of adhesion molecules and introduce a pathogenicity score to compare the relative effects of different antibodies. From this analysis, we showed that the biophysical response of keratinocytes to distinct PV antibodies is highly specific, allowing for accurate prediction of their pathogenicity. For instance, the high pathogenicity scores of the PVIgG and AK23 antibodies show strong agreement with their reported PV pathology. Our data-driven approach offers a detailed framework for the action of antibodies in pemphigus and paves the way for the development of effective diagnostic and therapeutic strategies.
寻常型天疱疮(PV)是一种影响皮肤和黏膜的水疱性自身免疫性疾病。PV抗体诱导角质形成细胞间黏附丧失的机制尚未完全明确。目前认为,该过程始于抗体与桥粒靶点结合,导致桥粒解体以及随后细胞间黏附的结构改变。桥粒分子的体外成像已被用于表征这一初始阶段。然而,图像分析在为我们提供关于抗体结合后生物物理变化的更深入知识方面仍有未开发的潜力。目前,在PV病理学中,尚未有来自免疫荧光图像的定量框架。在此,我们试图通过研究PV抗体对黏附分子和细胞骨架网络的影响,建立生物物理变化与抗体致病性之间的关联。具体而言,我们引入了一种数据驱动的方法来定量评估抗体处理后黏附分子的扰动。我们识别出了独特的成像特征,这些特征标志着抗体结合对黏附分子重塑的影响,并引入了致病性评分来比较不同抗体的相对效应。通过该分析,我们表明角质形成细胞对不同PV抗体的生物物理反应具有高度特异性,从而能够准确预测其致病性。例如,PVIgG和AK23抗体的高致病性评分与其报道的PV病理学表现高度一致。我们的数据驱动方法为天疱疮中抗体的作用提供了一个详细的框架,并为有效诊断和治疗策略的开发铺平了道路。