Ivanciu Lacramioara, Camire Rodney M
Division of Hematology and the Raymond G. Perelman Center for Cellular and Molecular Therapeutics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
IUBMB Life. 2025 May;77(5):e70024. doi: 10.1002/iub.70024.
Blood coagulation factor IX plays a crucial role in the intrinsic pathway of coagulation by generating factor Xa, ultimately leading to thrombin formation. Over the past 50 years, extensive research has deepened our understanding of the biology, physiology, pathology, biochemistry, and molecular genetics of factor IX. This wealth of knowledge has revealed how the factor IX gene and protein evolved, how factor IX is regulated, how it functions within the coagulation cascade, and how structural changes affect its function. In this review, we will summarize current knowledge on the biology of factor IX, with a focus on its structure-function relationships, gene structure, and regulation.
凝血因子IX通过生成因子Xa在凝血的内源性途径中发挥关键作用，最终导致凝血酶形成。在过去50年里，广泛的研究加深了我们对因子IX的生物学、生理学、病理学、生物化学和分子遗传学的理解。这些丰富的知识揭示了因子IX基因和蛋白质是如何进化的，因子IX是如何被调控的，它在凝血级联反应中是如何发挥作用的，以及结构变化如何影响其功能。在这篇综述中，我们将总结关于因子IX生物学的当前知识，重点关注其结构-功能关系、基因结构和调控。
