Stem Cell Transplantation Benefits Mice With Childhood Motor Neuron Disease

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Stem Cell Transplantation Benefits Mice With Childhood Motor Neuron

Disease

04 Sep 2008

The motor neuron disease spinal muscular atrophy (SMA) is the second

most common genetic disorder leading to death in childhood. There is

currently no cure for SMA, but some clinicians and researchers

consider stem cell transplantation as a potential therapeutic

strategy. And now, Giacomo Comi and colleagues, at the University of

Milan, Italy, have generated data using a mouse model of SMA to

suggest that spinal cord neural stem cells (NSCs) might be a possible

treatment for individuals with SMA.

In the study, NSCs from mice in which a green marker protein was

expressed only in nerve cells known as motor neurons (the cells that

are defective in SMA) were transplanted into the fluid bathing the

spinal cord of mice with an SMA-like disease. The transplanted cells

developed into a small number of motor neurons and the treated mice

showed improved muscular function and increased lifespan, when

compared with untreated mice. Further analysis indicated that the

major effect of NSC transplantation was that the transplanted cells

improved the survival and function of the motor neurons already in

the mice, making them more like normal motor neurons (at the gene

expression level). The authors therefore suggest that in the future,

NSCs might be used in the development of therapeutic protocols for

the treatment of SMA and other motor neuron diseases.

Notes:

TITLE: Neural stem cell transplantation can ameliorate the phenotype

of a mouse model of spinal muscular atrophy

AUTHOR:

Giacomo P. Comi

University of Milan, Milan, Italy.

http://www.unimi.it

View the PDF of this article at: https://www.the-jci.org/article.php?

id=35432

Source:

Honey

Journal of Clinical Investigation

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Article URL: http://www.medicalnewstoday.com/articles/120170.php