Effect of alpha-galactosidase A deficiency on FV leiden fibrin deposition and thrombosis in mice.
- Author:
Yue-Chun SHEN
1
;
Zhao-Chu HE
;
Ru-Li CAI
;
Jie-Zhen PAN
;
Xiao-Ming WANG
;
Jun LI
Author Information
- Publication Type:Journal Article
- MeSH: Animals; Fabry Disease; genetics; Factor V; genetics; Genotype; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mutation; Thrombosis; genetics; pathology
- From: Chinese Journal of Hematology 2009;30(3):162-165
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVETo evaluate the effect of alpha-galactosidase A (Gla) deficiency on FV Leiden (FVL) associated thrombosis in vivo.
METHODSTo generate the mice carrying mutations in Gla and FVL and analyze the tissue fibrin deposition in organs and thrombosis.
RESULTSIn 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].
CONCLUSIONSGla deficiency could be an important genetic modifier for the enhanced thrombosis associated with FVL.
