Complement factor B is a protein that in humans is encoded by the CFB gene.

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Complement factor B is a protein that in humans is encoded by the CFB gene.\n

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Function

This gene encodes complement factor B, a component of the alternative pathway of complement activation. It is directly paralogous to complement component 2 (C2) of the classical pathway, occupying the same locus on Chromosome 6 (6p21.33) and sharing 39% sequence homology, with even greater functional homology due to conservative amino acid replacements accounting for 11% of the divergence. Both are serine proteases with directly analogous functions in their respective complement activation pathways.\n

Factor B circulates in the blood as a single chain polypeptide. Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. The active subunit Bb harbors the serine protease domain that associates with C3b to form the alternative pathway C3 convertase. Bb is involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation. This gene localizes to the major histocompatibility complex (MHC class III) region on chromosome 6. This cluster includes several genes involved in regulation of the immune reaction. The polyadenylation site of this gene is 421 bp from the 5' end of the gene for complement component 2.\n

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References

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External links

GeneReviews/NCBI/NIH/UW entry on Atypical Hemolytic-Uremic Syndrome
OMIM entries on Atypical Hemolytic-Uremic Syndrome
The MEROPS online database for peptidases and their inhibitors: S01.196
Complement+Factor+B at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
Overview of all the structural information available in the PDB for UniProt: P00751 (Complement factor B) at the PDBe-KB.

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Function

References

Further reading

External links

Factor B

Broadcast History