摘要
纤维蛋白原(fibrinogen,Fg)作为凝血系统中含量最高的凝血因子,不仅是凝血系统的主要成分,也是一种重要的急性反应蛋白,参与体内多种生理与病理过程。研究发现,基因突变有可能导致纤维蛋白原的功能和代谢异常以及临床上出血、血栓症状的发生,与多种临床疾病密切相关。由纤维蛋白原缺陷引起的疾病主要分为获得性和遗传性2大类。本文将遗传性纤维蛋白原缺陷症的分子基础及其可能的作用机制、临床研究作一综述。
As the most abundant component of coagulation system, fibrinogen not only takes part in clotting, but also works as one of acute phase proteins, which participates in many physiological and pathophysiological processes. Studies of fibrinogen abnormalities contribute to understand the molecular basis of disorders of fibrinogen protein function and metabolism, caused mainly by gene mutation, commonly associated with bleeding, thrombophilia, or both. Diseases affecting fibrinogen could be classified to the acquired or inherited disease. In this review, the research progress on the molecular basis, possible action mechanism of the hereditary fibrinogen abnormalities and its clinical reasearch are summarized.
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2014年第4期1188-1192,共5页
Journal of Experimental Hematology
基金
梧州市科学研究与技术开发计划项目(201301047)
