Factor IXa

Structure

spinqualitylabelsall models Caption for this structure Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D The crystal structure of Factor IXa complexed with p-amino-benzamidine. Function The Factor IX protein is a serine protease that is part of the blood coagulation cascade. Disease Defects in the F9 gene, coding for Factor IX, result in the disease Hemophilia B. The F9 gene is located on the X chromosome. Hemophilia B is distinct from Hemophilia A, which results from defects in the F8 gene coding for Factor VIII, also located on the X chromosome. Factor IXa (the activated, clipped form of Factor IX) interacts with Factor VIIIa to form an active protease, which intern clips Factor X to its active form, Factor Xa. Structural highlights This is a sample scene created with SAT to color by Group, and another to make a transparent representation of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes. Here is an Overview of the Catalytic TriadThe catalytic triad at the active site is comprised of His57, Asp102 and Ser1985 is highlighted. Now Zoom in on the Catalytic Triad. The His borrows an H from Her, which now attacks at the substrate. In this case the active site is blocked by the inhibitor, p-amino-benzamidine. Mutation of C252S results in Hemophilia B, probably due to destabilization of the loop including the His of the catalytic triad. A mutation of T194A exhibits increased activity and stability and appears to enhance the risk for Deep Vein Thrombosis and stroke.

Proteopedia Page Contributors and Editors (what is this?)

James Nolan, Michal Harel