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Complement Factor B – Active Fragment: Bb

Molecular classification: Serine protease, Complement protein

Overview

Complement factor B (CFB) is a key protein in the alternative pathway of the complement system. The Bb fragment, generated by cleavage of factor B by factor D, is the active serine protease subunit. It forms the C3 convertase (C3bBb) and C5 convertase ((C3b)_2Bb), which are essential for amplifying the complement cascade. Mutations or dysregulation of Bb activity are linked to several diseases including aHUS, AMD and SLE.

Other names: CFBFactor B fragment BbBb fragment of complement factor B

Mechanism of action

Inhibition of serine protease activity of Bb, disruption of C3/C5 convertase formation

Biological functions

Immune responseComplement activationProteolysisB lymphocyte proliferation and differentiationMonocyte activation

Disease associations

Atypical hemolytic uremic syndrome (aHUS)Age-related macular degeneration (AMD)Systemic lupus erythematosus (SLE)InfectionInflammation

Safety considerations

Dysregulation of complement system can lead to autoimmune diseasesInhibition may increase susceptibility to infection

Biomarkers

Elevated levels of Bb in plasma may indicate inflammation or complement activation

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