[α-半乳糖苷酶A缺乏对小鼠FV莱顿纤维蛋白沉积和血栓形成的影响]

[Effect of alpha-galactosidase A deficiency on FV leiden fibrin deposition and thrombosis in mice].

作者信息

Shen Yue-Chun, He Zhao-Chu, Cai Ru-Li, Pan Jie-Zhen, Wang Xiao-Ming, Li Jun

机构信息

First Affiliated Hospital, Guangzhou Medical College, Guangzhou 510120, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2009 Mar;30(3):162-5.

PMID:19642363

Abstract

OBJECTIVE

To evaluate the effect of alpha-galactosidase A (Gla) deficiency on FV Leiden (FVL) associated thrombosis in vivo.

METHODS

To generate the mice carrying mutations in Gla and FVL and analyze the tissue fibrin deposition in organs and thrombosis.

RESULTS

In the presence of FVL, Gla deficiency greatly increased tissue fibrin deposition compared with that in wild-type [Gla(-/0) FV(Q/Q) vs. Gla(+/0) FV(Q/Q) = (0.24 +/- 0.07)% vs. (0.086 +/- 0.049)%, P < 0.0001; Gla(-/-) FV(Q/Q) vs. Gla(+/+) FV(Q/Q) = (0.32 +/- 0.03)% vs. (0.06 +/- 0.005)%, P < 0.05]. With Gla deficiency, the number of thrombi on organ sections in FVL mice was significantly increased [(Gla(-/-) FV(Q/Q) and Gla(-/0) FV(Q/Q)) vs. (Gla(+/+) FV(Q/Q) and Gla(+/0) FV(Q/Q)) = 1.9 +/- 0.7 vs. 0.3 +/- 0.1, P < 0.05].

CONCLUSIONS

Gla deficiency could be an important genetic modifier for the enhanced thrombosis associated with FVL.

摘要

目的

评估α-半乳糖苷酶A（Gla）缺乏对体内FV莱顿（FVL）相关血栓形成的影响。

方法

构建携带Gla和FVL突变的小鼠，并分析器官中的组织纤维蛋白沉积和血栓形成情况。

结果

在存在FVL的情况下，与野生型相比，Gla缺乏显著增加了组织纤维蛋白沉积[Gla(-/0) FV(Q/Q) 与Gla(+/0) FV(Q/Q) = (0.24 ± 0.07)% 与(0.086 ± 0.049)%，P < 0.0001；Gla(-/-) FV(Q/Q) 与Gla(+/+) FV(Q/Q) = (0.32 ± 0.03)% 与(0.06 ± 0.005)%，P < 0.05]。随着Gla缺乏，FVL小鼠器官切片上的血栓数量显著增加[(Gla(-/-) FV(Q/Q) 和Gla(-/0) FV(Q/Q)) 与(Gla(+/+) FV(Q/Q) 和Gla(+/0) FV(Q/Q)) = 1.9 ± 0.7 与0.3 ± 0.1，P < 0.05]。