C1QBP Human
Greater than 95.0% as determined by Analysis by SDS-PAGE.
Description
Product Specs
Product Science Overview
C1 is a multimolecular complex composed of three subcomponents:
- C1q: This is the recognition protein that binds to antibodies complexed with antigens or directly to pathogen surfaces.
- C1r: This is a serine protease that, upon activation, cleaves and activates C1s.
- C1s: This is another serine protease that cleaves the next components in the pathway, C4 and C2 .
The C1 complex is structured such that a single C1q molecule is bound to two molecules each of C1r and C1s, forming a C1q(C1r)₂(C1s)₂ complex .
The primary function of C1 is to initiate the classical pathway of complement activation. This process begins when C1q binds to the Fc region of antibodies (IgG or IgM) that are attached to antigens on the surface of pathogens. This binding induces a conformational change in the C1 complex, leading to the activation of C1r, which in turn activates C1s .
Once activated, C1s cleaves C4 into C4a and C4b. C4b then binds to the pathogen surface and subsequently binds C2, which is cleaved by C1s to form C2a and C2b. The C4bC2a complex, also known as C3 convertase, then cleaves C3 into C3a and C3b, leading to opsonization of the pathogen and further propagation of the complement cascade .
Recombinant human Complement Component 1 is produced using recombinant DNA technology, which involves inserting the gene encoding C1 into a suitable expression system, such as bacteria, yeast, or mammalian cells. This allows for the large-scale production of C1 for research and therapeutic purposes .
Deficiencies or dysfunctions in any of the components of C1 can lead to immune system disorders. For example, C1q deficiency is associated with autoimmune diseases such as systemic lupus erythematosus (SLE). Understanding the structure and function of C1 is crucial for developing therapeutic interventions for such conditions .
