Complement C4

spinqualitylabelsall models Glycosylated human complement C4-Aα chain (grey), C4 β (green) and γ chain (pink) (PDB code 4fxk) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Function Complement C4 (CC4) is part of the complement pathway. CC4 is a mixture of CC4-A and CC4-B. CC4 is part of the complement cascade which results in pathogen cell membrane pore and the eventual lysis of the pathogen cell. Disease C4 deficiency causes lupus-like autoimmunity. Copy number variations of complement C4 are associated with Behcet's disease. Level of complement C4 is associated with persistence and disease progression of chronic hepatitis C.

3D structures of complement C4

Updated on 29-November-2022

5jpn, 5jtw – hCC4a- human
1hzf – hCC4a residues 970-1336
5jpm – hCC4a + mannan-binding lectin serine protease
4xam – hCC4a residues 757-1744 + hCC4-B residues 20-675
4fxk – hCC4a α chain residues 680-1446 + hCC4 β residues 2—675 +γ residues 1454-1744 chains
4fxg – hCC4a α chain residues 680-1446 + hCC4 β residues 2—675 +γ residues 1454-1744 chains + mannan-binding lectin serine protease
6ysq – hCC4b + nanobody
7b2m, 7b2p, 7b2q – hCC4b + nanobody – Cryo EM

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Michal Harel, Joel L. Sussman