Treatment of Diseases Related to Hyperactivity of the Complement System

Abstract

Raising the level of Factor I above physiological levels can be used to treat diseases in which the underlying pathology is linked to overactivity of the C3b-feedback cycle and the generation and pro-inflammatory effects of iC3b. Methods, agents, and compositions for treatment of such diseases are described.

1 - 53 . (canceled) 
     
     
         54 . A method for treating Age-related Macular Degeneration (AMD) or atypical Hemolytic Uremic Syndrome (aHUS) in a human subject, the method comprising:
 administering a pharmaceutical composition comprising a therapeutically effective amount of an agent providing native Factor I, or a polypeptide that has at least 90% amino acid identity with native Factor I and that retains C3b-inactivating activity and iC3b degrading activity, to the human subject;   thereby treating AMD or aHUS in the human subject.   
     
     
         55 . The method according to  claim 54 , wherein the agent provides native Factor I, or a polypeptide that has at least 95% amino acid identity with native Factor I and that retains C3b-inactivating activity and iC3b degrading activity. 
     
     
         56 . The method according to  claim 54 , wherein the pharmaceutical composition is administered intravenously, intraocularly, intraperitoneally, intramuscularly, or intradermally. 
     
     
         57 . The method according to  claim 56 , wherein the pharmaceutical composition is administered intravenously or intraocularly. 
     
     
         58 . The method according to  claim 54 , wherein the pharmaceutical composition does not comprise Factor H. 
     
     
         59 . The method according to  claim 54 , wherein the method comprises lowering the amount of C3b activity in the subject. 
     
     
         60 . The method according to  claim 54 , wherein the level of C3b-inactivating and iC3b-degradation activity in the subject's plasma is increased. 
     
     
         61 . A method for treating Age-related Macular Degeneration (AMD) or atypical Hemolytic Uremic Syndrome (aHUS) in a human subject, the method comprising:
 administering a pharmaceutical composition comprising a therapeutically effective amount of an agent selected from native Factor I and a polypeptide that has at least 90% amino acid identity with native Factor I and that retains C3b-inactivating activity and iC3b degrading activity to the human subject;   thereby treating AMD or aHUS in the human subject.