Effect of alpha-galactosidase A deficiency on FV leiden fibrin deposition and thrombosis in mice / 中华血液学杂志

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the effect of alpha-galactosidase A (Gla) deficiency on FV Leiden (FVL) associated thrombosis in vivo.</p><p><b>METHODS</b>To generate the mice carrying mutations in Gla and FVL and analyze the tissue fibrin deposition in organs and thrombosis.</p><p><b>RESULTS</b>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].</p><p><b>CONCLUSIONS</b>Gla deficiency could be an important genetic modifier for the enhanced thrombosis associated with FVL.</p>