CD40-CD40L system in the immunopathogenesis of Viliuisk encephalomyelitis

Abstract

Viliuisk encephalomyelitis (VE) is a neurodegenerative disease that is common only among the indigenous inhabitants of Yakutia, in whose pathogenesis immunopathological processes play a significant role. VE patients demonstrate an increased level of soluble sCD40L ligand, expressed on immune cells and blood platelets, and which can affect the development of vascular pathology and inflammatory processes in the brain. It is suggested that metalloproteinase enzymes with increased protease activity can contribute to this process. The aim of the work was an experimental verification of the hypothesis of endoprotease activity of serum of VE patients. The results of the study during platelet incubation of a healthy donor with serum of VE patients confirmed the increase in the level of soluble sCD40L ligand in peripheral blood in VE patients due to protease activity. Increased activity in the blood of metalloproteinases that cleave sCD40L ligand from platelets can lead to microvascular pathology of the brain and be a major factor in the pathogenesis of primary chronic VE.