• Thus, C2 is an important component of both the classical and the lectin pathways of complement activation and is involved in first line defense against microbial infection that is essential for detection and clearance of the invading pathogens [ 6 ]. (biomedcentral.com)
  • The complement system has three independent pathways of complement activation: a classical pathway, an alternative pathway, and a lectin pathway. (biomedcentral.com)
  • Complements are activated in three independent pathways: a classical pathway, an alternative pathway, and a lectin pathway (Fig. 1 ). (biomedcentral.com)
  • This has resulted in achieving important information on the role of complement as a major mediator and effector mechanism in diseases of immune and non-immune pathogenesis. (biomedcentral.com)
  • The complement system is a major effector of humoral immunity and natural immunity. (biomedcentral.com)
  • Complement component C2 functions as a key regulator in the early activation phase of the classical pathway and participates in the formation of the classical pathway C3 convertase C4b2a [ 4 ]. (biomedcentral.com)
  • The classical pathway is triggered when the complement C1 complex binds to antibodies that are bound to antigens. (biomedcentral.com)
  • Complement component 4 (C4), in humans, is a protein involved in the intricate complement system, originating from the human leukocyte antigen (HLA) system. (wikipedia.org)
  • The complement system consists of more than 30 soluble and membrane proteins and constitutes an important mediator of host defense against foreign pathogens. (biomedcentral.com)
  • The alternative activation pathway, which is C3 dependent, is generally intact in C2 deficiency and can trigger formation of the membrane attack complex (MAC) independently of C2 [ 4 ]. (biomedcentral.com)
  • This pathway also leads to the formation of various bioactive molecules such as C5a and the formation of membrane attack complex on the surface of target cells. (biomedcentral.com)
  • Complement activation results in the formation of membrane attack complex (MAC), and this complex produces holes in the cell membrane, causing the destruction of target cells. (biomedcentral.com)
  • The activation of this cascade culminates in the generation of various fragments derived from complement proteins and the formation of membrane attack complex (MAC). (biomedcentral.com)
  • In the process, C4b and C2a are produced, and these two fragments combine to form C3 convertase. (biomedcentral.com)
  • For example, the severity of a dysfunctional complement system can lead to fatal diseases and infections. (wikipedia.org)
  • The complement system consists of more than 30 proteins. (biomedcentral.com)
  • Complement C2 deficiency is the most common genetically determined complete complement deficiency and is associated with a number of diseases. (biomedcentral.com)
  • The links with these diseases reflect the important role complement C2 plays in both innate immunity and immune tolerance. (biomedcentral.com)
  • Complement is not only important for protection against microorganisms, but also contributes to the pathophysiology of a number of autoimmune diseases. (biomedcentral.com)
  • Genetic deficiencies of complement components are a common denominator of immune and infectious diseases. (biomedcentral.com)
  • Anti-complement agents have garnered attention as a new treatment strategy for refractory inflammatory diseases. (biomedcentral.com)
  • Complement fragments such as C3a, C4a, and C5a trigger inflammation as anaphylatoxins and chemotactic factors, and abnormal complement activation leads to various inflammatory diseases [ 1 , 2 ]. (biomedcentral.com)
  • Over the past few years, a monoclonal antibody against complement component C5 (eculizumab) has been approved as a treatment for paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome (aHUS), and this antibody has yielded encouraging results [ 3 , 4 ]. (biomedcentral.com)
  • Once factor D is activated, factor B and then complement component C3 are activated. (biomedcentral.com)
  • However, in the absence of C2, C3 is, in many situations, not efficiently cleaved resulting in a limited deposition of C3 fragments on immune complexes and on the surface of apoptotic cells. (biomedcentral.com)
  • More specifically, the two proteins, Ch and Rg, function together as a medium for interaction between the Ab-Ag complex and other complement components. (wikipedia.org)
  • Human complement C2 was cloned and expressed in a mammalian cell line. (biomedcentral.com)
  • Taken together these data suggest that recombinant human C2 can restore classical complement pathway activity and may serve as a potential therapeutic for recurring bacterial infections or SLE in C2-deficient patients. (biomedcentral.com)
  • Our understanding of the role of complement in human disease is the result of numerous studies in recent years focused on complement's mechanism of action. (biomedcentral.com)