In vivoでは、第XII因子はポリアニオンとの接触によって活性化される。活性化された血小板はポリリン酸などの無機ポリマーを分泌する。ポリリン酸との接触は第XII因子を活性化し、血栓形成に重要な内因性経路を介してフィブリンの形成を開始する。ホスファターゼによるポリリン酸の標的化によって、マウスでは活性化された血小板の凝固促進活性の干渉がみられ、血小板によって誘導される血栓形成が阻害された。ヘルマンスキー・パドラック症候群(英語版)の患者でみられる血漿凝固の欠陥はポリリン酸の付加によって回復し、このことはこの無機ポリマーがin vivoでの内在性第XII因子の活性化因子であることを示している。血小板のポリリン酸にって駆動される第XII因子の活性化は、一次止血(血小板血栓(英語版)の形成)から二次止血(フィブリンメッシュの形成)への接続を担う[13]。
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関連文献
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