[Effect of transplantation of wild-type bone marrow stem cells in mouse model of familial amyotrophic lateral sclerosis].
OBJECTIVE
To determine the effects of wild-type mouse bone marrow stem cells transplants on survival time and motor functions in the human mutant SOD1-G93A mouse model of familial amyotrophic lateral sclerosis (ALS).


METHODS
Bone marrow stem cells derived from the wild-type male mice were delivered intravenously into 25 ALS transgenic female mice (carrying the human SOD1 gene with Gly 93 Ala mutation) that had been pretreated with 5.5-6.5 Gy gamma-ray 5-7 days before. The onset time of limbs paralysis, lifespan and the graft versus host disease (GVHD) symptoms were observed in the treated group and statistically compared with control group of 15 media-injected ALS transgenic mice. The Sry gene (sex determine region on the Y chromosome) were detected by polymerase chain reaction technique in the blood sample of treated female ALS mice after 8 weeks of transplantation. A series of animal motor tests including rotating rods, rotated wheel and extension reflex were performed in both two groups at the same age of 16-17 weeks to assess the mice survival motor functions. Results A few treated mice (7/25) had different clinical presentations of GVHD. The semi-quantity evaluation score of average GVHD among the treated ALS mice was not over 1-2. The detection of Sry gene on these treated female ALS group was positive. The average onsets of limb paralysis and survival time were prolonged for about 5 weeks. At the age of 16-17 weeks, the motor function in the treated group was significantly better than in the ALS control group (P < 0.01).


CONCLUSIONS
Transplantation of wild-type mice bone marrow stem cells can prolong survival in the recipient mice and ameliorate motor dysfunction. Intravenous administration of normal bone marrow stem cells may have therapeutic values for ALS.
Huang
Hui
H
Department of Neurology, First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China.
Zhang
Cheng
C
Zhao
Cui-Ping
CP
Yao
Xiao-Li
XL
Xi
Jing
J
D000690:Amyotrophic Lateral Sclerosis / Q000601:surgery*; D000818:Animals; D005260:Female; D006086:Graft vs Host Disease / Q000188:drug therapy; D008297:Male; D045164:Mesenchymal Stem Cell Transplantation; D051379:Mice; D008822:Mice, Transgenic; D015996:Survival Rate