FGG 基因的新型突变导致一个中国家族出现低纤维蛋白原血症。

IF 2.7 3区 生物学 Hereditas Pub Date : 2024-02-20 DOI:10.1186/s41065-024-00313-3
Xiaoying Xie, Juan Du, Shunkang Geng, Baoqin Yi, Qingpu Li, Jiangcheng Zuo
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引用次数: 0

摘要

先天性纤溶酶原异常是由纤溶酶原缺陷引起的一组凝血功能障碍,分为四种类型,包括低纤溶酶原血症、低纤溶酶原血症、纤溶酶原异常血症和低纤溶酶原血症。本研究收集了一个低纤维蛋白原血症家族,并通过遗传学分析确定了 FGG 基因中的一个新的致病变体(c.668G > C, p.Arg223Thr)。电子显微镜观察显示,该患者的纤维蛋白超微结构发生了显著变化。我们的研究拓展了与 FGG 基因相关的表型和遗传谱,有助于遗传咨询和产前遗传诊断。
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A novel mutation in the FGG gene causes hypofibrinogenemia in a Chinese family.

Congenital fibrinogen disorders are a group of coagulation deficiencies caused by fibrinogen defects and are divided into four types, including afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia. In this study, we collected a family with hypofibrinogenemia, and genetics analysis identify a novel pathogenic variants (c.668G > C, p.Arg223Thr) in the FGG gene. And electron microscope observation revealed significant changes in the ultrastructure of fibrin of the proband. Our research expands the phenotypic and genetic spectrum associated with the FGG gene, which would facilitate in genetic counselling and prenatal genetic diagnosis.

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来源期刊
Hereditas
Hereditas Biochemistry, Genetics and Molecular Biology-Genetics
CiteScore
3.80
自引率
3.70%
发文量
0
期刊介绍: For almost a century, Hereditas has published original cutting-edge research and reviews. As the Official journal of the Mendelian Society of Lund, the journal welcomes research from across all areas of genetics and genomics. Topics of interest include human and medical genetics, animal and plant genetics, microbial genetics, agriculture and bioinformatics.
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