title: [Effect of alpha-galactosidase A deficiency on FV leiden fibrin deposition and thrombosis in mice].
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.
authors.affiliation: First Affiliated Hospital, Guangzhou Medical College, Guangzhou 510120, China.
authors.forename: Yue-Chun
authors.initials: YC
authors.lastname: Shen
authors.forename: Zhao-Chu
authors.initials: ZC
authors.lastname: He
authors.forename: Ru-Li
authors.initials: RL
authors.lastname: Cai
authors.forename: Jie-Zhen
authors.initials: JZ
authors.lastname: Pan
authors.forename: Xiao-Ming
authors.initials: XM
authors.lastname: Wang
authors.forename: Jun
authors.initials: J
authors.lastname: Li
mesh_terms: D000818:Animals; D000795:Fabry Disease / Q000235:genetics*; D005165:Factor V / Q000235:genetics*; D005838:Genotype; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009154:Mutation; D013927:Thrombosis / Q000235:genetics* / Q000473:pathology
chemical_list: C095381:factor V Leiden; D005165:Factor V