ДЕМОПУЛОС Грегори А. (US),ДАДЛЕР Том (US),ШВЕБЛЕ Ханс-Вильхельм (GB)
申请号：
RU2013149792/15
公开号：
RU2013149792A
申请日:
2012.04.06
申请国别(地区):
RU
年份:
2015
代理人:
摘要：
1. The use of a MASP-2 inhibitory agent that inhibits MASP-2-dependent complement activation in the manufacture of a medicament for the treatment of a subject suffering from paroxysmal nocturnal hemoglobinuria (PNH) .2. The use of claim 1, wherein the MASP-2 inhibitory agent is an anti-MASP-2 antibody or fragment thereof. The use of claim 2, wherein the MASP-2 inhibitory agent is a monoclonal anti-MASP-2 antibody or fragment thereof that specifically binds to a portion of SEQ ID NO: 6.4. The use of claim 1, wherein the MASP-2 inhibitory agent enhances the survival of red blood cells in a subject. The use of claim 1, wherein the subject exhibits one or more symptoms selected from the group consisting of (i) lower than normal hemoglobin levels, (ii) lower than normal platelet levels； (iii) higher reticulocyte levels than normal； and (iv) higher bilirubin levels than normal. 6. The use of claim 1, wherein the subject has previously received or is currently receiving treatment with a terminal complement inhibitor that inhibits cleavage of the C5 complement protein. The use of claim 1, wherein the drug is administered in conjunction with a terminal complement inhibitor that inhibits cleavage of the C5 complement protein. The use of claim 7, wherein the terminal complement inhibitor is a humanized anti-C5 antibody or antigen-binding fragment thereof. The use of claim 8, wherein the terminal complement inhibitor is eculizumab. 10. The use of claim 2, wherein the antibody or fragment thereof is selected from the group consisting of a recombinant antibody, an antibody with attenuated effector function, a chimeric antibody, humanis1. Применение MASP-2-ингибирующего агента, который ингибирует MASP-2-зависимую активацию комплемента, при изготовлении лекарственного препарата для лечения субъекта, страдающего от пароксизмальной ночной гемоглобинурии (PNH).2. Применение по п. 1, где MASP-2-ингибирующий агент представляет собой антитело против MASP-2 или его фрагмент.3. Применение
