An extended mini-complement factor H molecule ameliorates experimental C3 glomerulopathy.

ABSTRACT: Abnormal regulation of the complement alternative pathway is associated with C3 glomerulopathy. Complement factor H is the main plasma regulator of the alternative pathway and consists of 20 short consensus repeat (SCR) domains. Although recombinant full-length factor H represents a logical treatment for C3 glomerulopathy, its production has proved challenging. We and others have designed recombinant mini-factor H proteins in which 'non-essential' SCR domains have been removed. Here, we report the in vitro and in vivo effects of a mini-complement factor H protein, FH^{1-5^18-20}, using the unique factor H-deficient (Cfh-/-) mouse model of C3 glomerulopathy. FH^{1-5^18-20} is comprised of the key complement regulatory domains (SCRs 1-5) linked to the surface recognition doma

SUBMITTER: Nichols EM 

PROVIDER: S-EPMC4650264 | biostudies-literature | 2015 Dec

REPOSITORIES: biostudies-literature