C1q: its functions within the innate and adaptive immune responses and its role in lupus autoimmunity.
The complement cascade is a multi-faced effector component of the innate immune response. C1q is the recognition component of the classical pathway of complement activation. In addition, C1q has been recognized to serve a number of other biological functions including a modulating role on cellular functions within the adaptive immune response. The importance of C1q to normal immune regulation is reflected by the fact that greater than 90% of individuals who have complete congenital deficiency of C1q have been observed to develop early-onset photosensitive systemic lupus erythematosus (SLE). As a number of single nucleotide polymorphisms have been identified in three C1q genes, it is possible that more subtle variations in C1q expression could be a risk factor for cutaneous LE and SLE. Thus, a more comprehensive delineation of complotype could be of increasing clinical importance in the future.
Sontheimer
Richard D
RD
Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA. sontheimer@ouhsc.edu
Racila
Emil
E
Racila
Doina M
DM
D003167:Complement Activation; D015922:Complement C1q / Q000378:metabolism*; D015870:Gene Expression; D020022:Genetic Predisposition to Disease; D006801:Humans; D007110:Immunity, Active; D007113:Immunity, Innate; D008178:Lupus Erythematosus, Cutaneous / Q000276:immunology*; D008180:Lupus Erythematosus, Systemic / Q000276:immunology*; D020641:Polymorphism, Single Nucleotide; D012307:Risk Factors
D015922:Complement C1q