[Bone marrow stem cells transplantation improve locomotive function of dystrophin/utrophin gene double knock-out mice].
OBJECTIVE
Study the improvement of locomotive faculty of dystrophin/utropin gene double knock-out mice (dko mice) by transplanting bone marrow stem cells.


METHODS
The bone marrow stem cells of C57BL/6 mice (4- to 5-weeks age) were cultured in vitro for three days, before transplanted intravenously (1.0 x 10(7) for each) into 11 dko mice (7- to 8-weeks age). The dko mice were irridiated with 7Gy gamma-ray before transplantation. 8-9 weeks after transplantation, the locomotroy function, electromyography items and expression of dystrophin in transplanted mice and controls were observed.


RESULTS
8-9 weeks after transplantation, the dropping times of hauling wire were 3.09 +/- 2.47, compared with that of the control dko mice(16.78 +/- 3.60), there are distinct differences. About electromyography items, the duration of active potential and amplitude of maxim contractions were (4.99 +/- 1.62) ms and(2872 +/- 1474.33) microV, compare with those of control dko mice(3.69 +/- 0.40) ms and(1210.0 +/- 551.0) microV, respectively, about 7% fibers of the muscle tissue of transplanted dko mice expressed dystrophin protein.


CONCLUSIONS
8-9 weeks after transplanted with homology bone marrow stem cells, the locomotive function and electromyography items of transplanted dko mice were obviously improved, and about 7% muscle tissue fibers of the mice expressing dystrophin protein were observed. It suggested that there is an ideal prospect for DMD therapy with bone marrow stem cells transplantation.
Zhang
Cheng
C
Department of Neurology, First Affiliated Hospital of Sun Yat-sen, Guangzhou, 510080, China. CZYM@GZSUMS.EDU.CN
Chen
Song-lin
SL
Liu
Xiao-rong
XR
Huang
Wen
W
Zhang
Wei-xi
WX
Lu
Xi-lin
XL
D000818:Animals; D003598:Cytoskeletal Proteins / Q000096:biosynthesis / Q000172:deficiency / Q000235:genetics*; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics*; D018380:Hematopoietic Stem Cell Transplantation; D008565:Membrane Proteins / Q000096:biosynthesis / Q000235:genetics*; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009043:Motor Activity; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology / Q000601:surgery*; D049411:Utrophin
D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; C483600:Utrn protein, mouse; D049411:Utrophin