COMPOSITIONS AND METHODS OF INHIBITING MASP-1, MASP-2 AND/OR MASP-3 FOR TREATMENT OF PAROXYSMAL NOCTURNAL HEMOGLOBINURIA Russian patent published in 2018 - IPC A61K39/395 C07K16/40 A61P13/02 A61P7/00 

FIELD: medicine; pharmacology.

SUBSTANCE: invention relates to pharmacology and medicine. Disclosed is use of a composition comprising an amount of an agent inhibiting MASP-3, effective for inhibiting MASP-3-dependent complement activation, in the manufacture of a medicament for the treatment of an individual suffering from paroxysmal nocturnal hemoglobinuria (PNH), wherein the MASP-3 inhibitory agent is a monoclonal antibody against MASP-3 or a fragment thereof specifically binding to a portion of human MASP-3 (SEQ ID NO: 8). Technical result is that it is shown that the deficiency of MASP-3 reduces the percentage of complement-mediated lysis of mannose-coated red blood cells compared to normal human serum; the addition of an anti-MASP-3 antibody (e. g., mAbD14) to the serum of a patient with MASP-3 deficiency (ZMC) inhibits the alternatively triggered C3b accumulation and reduces the lysis of red blood cells.

EFFECT: technical result is that it is shown that the deficiency of MASP-3 reduces the percentage of complement-mediated lysis of mannose-coated red blood cells compared to normal human serum.

9 cl, 47 dwg, 19 ex, 19 tbl