F8-KO

品系全名

B6.129S-F8tm1Smoc

目录号

NM-KO-00012

品系状态

活体

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基因信息

基因名
F8

基因曾用名

Cf8; Cf-8; FVIII

NCBI ID

14069

MGI ID

88383

Ensembl ID

ENSMUSG00000031196

Pubmed

F8

人类同源基因

F8

品系描述

血友病A是由凝血因子VIII（FVIII）缺乏和(或)其功能障碍所引起的X连锁隐性遗传性出血病。FVIII基因敲除小鼠模型（简称“血友病A小鼠模型”），该小鼠模型具有明确的血友病A症状。该小鼠模型的建立为我国血友病A治疗药物的筛选和评价，以及血友病A的基因治疗研究提供了理想的动物模型和研究手段，具有重要的应用价值。饲养时需注意同笼小鼠间争斗可能导致小鼠内出血甚至死亡，剪尾后必须采取及时的止血措施如烧灼尾部切口，防止敲除纯合子小鼠失血和死亡。 通过敲除F8基因exon 16-19，建立F8-KO小鼠模型。与此相似的品系还有F8-KO(2)(NM-KO-200608),敲除区域为exon 1-26。
应用领域：血友病、F8因子缺失相关凝血机制研究和药物试验

疾病预测

血友病
Hemophilia

近似模型的表型

MGI:2449544

参考文献

Bi L, Lawler AM, Antonarakis SE, High KA, Gearhart JD, Kazazian HH Jr, Targeted disruption of the mouse factor VIII gene produces a model of haemophilia A [letter]. Nat Genet. 1995 May;10(1):119-21

验证数据

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Fig1. FVIII基因敲除小鼠构建策略示意图

4.png

Fig2. 不同品系F8敲除小鼠活化部分凝血活酶时间（APTT）检测。结果显示F8-KO小鼠（NM-KO-00012）凝血所需时间明显长于野生型小鼠，且F8-KO（NM-KO-00012）与F8-KO(2)（NM-KO-200608）凝血时间无明显差别，表型类似。


FVIII基因敲除小鼠是在整体动物水平研究血友病A发病机制和药物筛选的理想动物模型，可以为血友病A治疗药物的筛选和评价、血友病A的基因治疗等研究提供理想的动物模型和新的实验手段。为进一步阐述疾病发生机制、疾病防治药物的评价筛选和新疗法的研发提供了有效工具。

发表文献 3篇

1. Treatment of hemophilic arthropathy by immunomodulatory extracellular vesicle delivered by liposome hybrid nanoparticles
发表年份：2024 来源杂志：Bioactive Materials

2. Activated factor X targeted stored in platelets as an effective gene therapy strategy for both hemophilia A and B
发表年份：2021 来源杂志：Clinical and Translational Medicine

3. Alginate hydrogel-coated syringe needles for rapid haemostasis of vessel and viscera puncture
发表年份：2020 来源杂志：BIOMATERIALS

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