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    For Research Use Only. Not For Clinical Use.


    Background

    This gene encodes coagulation factor XI of the blood coagulation cascade. This protein is present in plasma as a zymogen, which is a unique plasma coagulation enzyme because it exists as a homodimer consisting of two identical polypeptide chains linked by disulfide bonds. During activation of the plasma factor XI, an internal peptide bond is cleaved by factor XIIa (or XII) in each of the two chains, resulting in activated factor XIa, a serine protease composed of two heavy and two light chains held together by disulfide bonds. This activated plasma factor XI triggers the middle phase of the intrisic pathway of blood coagulation by activating factor IX. Defects in this factor lead to Rosenthal syndrome, a blood coagulation abnormality. [provided by RefSeq, Jul 2008]
    Protein class

    Candidate cardiovascular disease genes, Disease related genes, Enzymes, FDA approved drug targets, Human disease related genes, Plasma proteins

    Predicted location

    Intracellular, Secreted (different isoforms)

    Single cell type specificity

    Group enriched (Hepatocytes, Alveolar cells type 2)

    Immune cell specificity

    Not detected in immune cells

    Cell line specificity

    Cell line enriched (Hep G2)

    Interaction

    Homodimer; disulfide-linked. Forms a heterodimer with SERPINA5. After activation the heavy and light chains are also linked by a disulfide bond.

    Molecular function

    Heparin-binding, Hydrolase, Protease, Serine protease

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