Abstract

THE STRATEGY FOR IDENTIFYING FUNCTIONAL ACTIVITIES OF THE PATIENT COMPLEMENT SYSTEM EARLY COMPONENTS LINKED INTO THE BLOOD SUPRAMOLECULAR COMPLEXES OF PATHOLOGICAL ORIGIN: CHEMILUMINESCENT ANALYSIS OF FUNCTIONAL ACTIVITY OF THE С1- INHIBITOR OF PATIENTS

Lakhtin V. M.*, Lakhtin M. V. and Aleshkin V. A.

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Abstract

A strategy for conducting chemiluminescent analysis (CLA) of the functional activity of the complement early components is proposed. Based on the strategy, CLA has been developed in relation to the C1 inhibitor (C1-inh) of patients with autoimmune diseases and complement C4 component deficiency. The zone pI 4-5 of pathological aggregation of proteins with antigens of the complement components was determined as corresponding to the blotting CLA developed by us to establish the regions of the C4A isotype (enriched mainly with a protein component) and the C4B isotype (more enriched with carbohydrates/ glycans/ glycoconjugates against the background of the presence of protein). The appearance of the C1-inh antigen in the zone indicates its consumption (the appearance of a functional deficiency of the complement component) in the blood with the formation of complex protein and non-protein aggregates with the C1-inh antigen that are not excreted from the bloodstream. In the case of a genetically determined deficiency of C4B in patients (and, as a result, the absence of C4B in the expected area in CLA), the appearance of complexes with C1-inh in the area of C4B location indicates: joint localization of C1-inh and C4B (linked participation of C1-in with a C4B-dependent complement cascade); the replacement of hereditary C4B deficiency by the participation of C1-inh in C4B-independent cascades of reactions with the result of countering the disease. General prospects for proposed strategy of further using CLA are represented.

Keywords: C1-inhibitor, C4B and C4A, biomarker, functional activity, complement component deficit, isoelectric focusing in a polyacrylamide gel, immunoblotting, Durapore membrane, Immobillon P, chemiluminescence in a live imagination, BioWest, BioChemi System, auto