Paronyan Adrine, Muradyan Nelli, Sargsyan Arsen, Arakelov Vahram, Arakelov Grigor, Nazaryan Karen
Institute of Molecular Biology of the National Academy of Sciences of the Republic of Armenia (NAS RA), Yerevan 0014, Armenia.
Russian-Armenian (Slavonic) University, Yerevan 0051, Armenia.
ACS Omega. 2025 Jan 23;10(4):3462-3473. doi: 10.1021/acsomega.4c07386. eCollection 2025 Feb 4.
Familial Mediterranean fever (FMF) is a genetically determined disease transmitted through autosomal recessive inheritance. Recently, a rare but similar disease to FMF, pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND), has been discovered. PAAND is inherited dominantly and is characterized by neutrophilic dermatosis and recurrent fevers. The cause of the disease is point mutations in the MEFV gene. The pyrin protein is a product of this gene and is one of the main factors in the disease's progression. This paper examines the interaction between pyrin and the 14-3-3 protein and screens for modulators affecting their interaction. Regulating this interaction is crucial for understanding the mechanism of FMF development, specifically the disruption of this complexation, which leads to inflammatory responses. The pyrin-14-3-3 interaction is essential for designing potential drugs since weakening this interaction can result in inflammation. This research of in silico experiments identified low molecular weight chemical compounds that have a modulating effect on the tertiary structures of mutant variations of pyrin and 14-3-3 proteins. Studies have identified modulator molecules that interact with the FMF-associated mutant structure of pyrin (M694I) and 14-3-3τ. The chemical compounds that result from this process can be used as modulators and as a potential new basis for the development of therapeutic drugs.
家族性地中海热(FMF)是一种通过常染色体隐性遗传传播的基因决定疾病。最近,一种与FMF罕见但相似的疾病——伴有嗜中性皮病的吡啉相关自身炎症(PAAND)被发现。PAAND以显性方式遗传,其特征为嗜中性皮病和反复发热。该疾病的病因是MEFV基因中的点突变。吡啉蛋白是该基因的产物,是疾病进展的主要因素之一。本文研究了吡啉与14-3-3蛋白之间的相互作用,并筛选影响它们相互作用的调节剂。调节这种相互作用对于理解FMF的发病机制至关重要,特别是这种复合物形成的破坏会导致炎症反应。由于减弱这种相互作用会引发炎症,因此吡啉-14-3-3相互作用对于设计潜在药物至关重要。这项计算机模拟实验研究确定了对吡啉和14-3-3蛋白突变变体的三级结构具有调节作用的低分子量化合物。研究已经确定了与吡啉(M694I)和14-3-3τ的FMF相关突变结构相互作用的调节分子。这一过程产生的化合物可用作调节剂,并作为治疗药物开发的潜在新基础。