Effect of intrastriatal mesenchymal stromal cell injection on progression of a murine model of Krabbe disease

被引：17
作者：
Wicks, Shawna E.
Londot, Heaven
Zhang, Bo [1 ]
Dowden, Jennifer
Klopf-Eiermann, Jessica [2 ]
Fisher-Perkins, Jeanne M. [2 ]
Trygg, Cynthia B. [2 ]
Scruggs, Brittni A. [3 ,4 ]
Zhang, Xiujuan [4 ]
Gimble, Jeffrey M. [1 ]
Bunnell, Bruce A. [2 ,3 ,4 ]
Pistell, Paul J.
机构：
[1] Pennington Biomed Res Ctr, Stem Cell Biol Lab, Baton Rouge, LA 70808 USA
[2] Tulane Natl Primate Res Ctr, Div Regenerat Med, Covington, LA 70433 USA
[3] Tulane Univ, Sch Med, Dept Pharmacol, New Orleans, LA 70112 USA
[4] Ctr Stem Cell Res & Regenerat Med, Sch Med, New Orleans, LA 70112 USA
来源：
BEHAVIOURAL BRAIN RESEARCH | 2011年 / 225卷 / 02期
关键词：
Krabbe disease; Twitcher mice; Mesenchymal stem/stromal cells; Adipose-derived stem/stromal cells; Gait analysis; Motor function; BONE-MARROW-TRANSPLANTATION; TWITCHER MOUSE; STEM-CELLS; CORD BLOOD; LEUKODYSTROPHY; THERAPY; DEMYELINATION; MICE; DIFFERENTIATION; SURVIVAL;
D O I：
10.1016/j.bbr.2011.07.051
中图分类号：
B84 [心理学]; C [社会科学总论]; Q98 [人类学];
学科分类号：
03 ; 0303 ; 030303 ; 04 ; 0402 ;
摘要：
One of a family of devastating lysosomal storage disorders, Krabbe disease is characterized by demyelination, psychosine accumulation, and inflammation. Affected infants rarely survive longer than 2 years. Using the twitcher mouse model of the disease, this study evaluated the potential of intrastriatal injection of adipose or bone marrow-derived mesenchymal stromal cells (MSCs) as a treatment option. Neonatal pups were injected with MSCs at 3-4 days of age and subjected to a battery of behavioral tests beginning at 15 days. While MSC injection failed to increase lifespan of twitchers, improvements in rotarod performance and twitching severity were observed at 27-38 days of age using MSCs derived from bone marrow. This study tested several different tasks developed in adult mice for evaluation of disease progression in immature twitchers. Rotarod was both reliable and extremely sensitive. Automated gait analysis using the Treadscan program was also useful for early evaluation of differences prior to overt gait dysfunction. Finally, this study represents the first use of the Stone T-maze in immature mice. Validation of rotarod and automated gait analysis for detection of subtle differences in disease progression is important for early stage efforts to develop treatments for juvenile disorders. (C) 2011 Elsevier B.V. All rights reserved.
引用
收藏
页码：415 / 425
页数：11
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