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CFB
CFB Full Name
complement factor B
CFB Introduction
Complement factor B is a protein that in humans is encoded by the CFB gene.
Figure. Structure of CFB.
Component factor B is a single chain peptide with a molecular weight of 93 kDa. Its binding with C3b makes the protein easily hydrolyzed and cleaved by factor D protein. The smaller cleavage product, released Ba fragment (33 kDa), is represented by three short shared repeat (SCR) domains, each encoded by a separate exon. The larger Bb fragment (60kDa) is encoded by 13 exons and contains domains homologous to other serine protease catalytic chains. Each functional domain of the active site is contained in a separate exon. The complement component factor B, which replaces the complement pathway, shares structural and functional homology with the classical pathway regulatory factor C2.
Factor B Deficiency and Related Diseases
CFB deficiency is a rare genetic disorder that impairs the alternative complement pathway, leading to increased susceptibility to infection and potential autoimmune or inflammatory complications. CFB deficiency is caused by mutations in the CFB gene that disrupt the ability of the alternative pathway to form the enzyme C3 convertase (C3bBb).
CFB deficiency may exhibit a number of clinical manifestations.
Recurrent infections: Increased susceptibility to Neisseria meningitidis (meningococcal infections) and Neisseria gonorrhoeae, and susceptibility to infections such as pneumococcal pneumonia or sepsis.
Autoimmune and inflammatory diseases: Dysregulation of complement activation may lead to autoimmune tissue damage, as in SLE.
C3 glomerulopathy (C3G): Autoantibodies that stabilize the C3 converting enzyme (e.g., anti-factor B autoantibodies) can lead to kidney damage.
Infectious shock: Impaired bacterial clearance exacerbates systemic inflammation.
Alzheimer's disease and multiple sclerosis: Chronic complement activation may lead to neuroinflammation.
Alternate Names for CFB
CFB; complement factor B; BF; FB; BFD; GBG; CFAB; CFBD; PBF2; AHUS4; FBI12; H2-Bf; ARMD14; C3 proactivator; C3/C5 convertase; C3 proaccelerator; properdin factor B; B-factor, properdin; glycine-rich beta glycoprotein; glycine-rich beta-glycoprotein;
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