MeSH definition : A 53-kDa protein that is a positive regulator of the alternate pathway of complement
activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE
PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating
the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX.
Individuals with mutation in the PFC gene exhibit properdin deficiency and have a
high susceptibility to infections.;
A 53-kDa protein that is a positive regulator of the alternate pathway of complement
activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE
PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating
the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX.
Individuals with mutation in the PFC gene exhibit properdin deficiency and have a
high susceptibility to infections.
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=2966 France French rare diseases properdin immunologic deficiency syndromes scientific and technical information