PMID: 20124762Feb 4, 2010Paper

Human umbilical cord blood-derived mesenchymal stem cells improve neurological abnormalities of Niemann-Pick type C mouse by modulation of neuroinflammatory condition

The Journal of Veterinary Medical Science
Hyun LeeHee Kyung Jin

Abstract

Niemann-Pick type C (NP-C) disease is a devastating developmental disorder with progressive and fatal neurodegeneration. We have used a mouse model of Niemann-Pick type C (NP-C) disease to evaluate the effects of direct intracerebral transplantation of human umbilical cord blood-derived mesenchymal stem cells (hUCB-MSCs) on the progression of neurological disease in this order. Here, we show that hUCB-MSCs transplantation into NP-C mice prevents the loss of Purkinje neurons and inhibits cerebellar apoptotic cell death. Interestingly, these effects were associated with the modulation of inflammatory responses, as evidenced by increased anti-inflammatory cytokine IL-10, and reduced abnormal astrocytic activation. Furthermore, our results show that the hUCB-MSCs transplantation reduced the cholesterol accumulation level in neurons in NP-C mice compared with sham-transplanted animals. This study provides the first evidence that hUCB-MSCs can improve neurological symptoms in NP-C disease, suggesting it as a potential therapeutic agent against neurodegenerative diseases.

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Citations

Nov 16, 2010·Annual Review of Pathology·Nora G Singer, Arnold I Caplan
May 25, 2011·World Journal of Stem Cells·Karen Bieback, Irena Brinkmann
Sep 16, 2010·Annals of the New York Academy of Sciences·Xiao Yan ZhongWolfgang Holzgreve
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Nov 26, 2010·Immunology and Cell Biology·Irene BergwerfPeter Ponsaerts
Dec 2, 2020·International Journal of Molecular Sciences·Valentina Pallottini, Frank W Pfrieger
Jul 6, 2019·Biochimica Et Biophysica Acta. Molecular Basis of Disease·Arubala P ReddyNurgul Carkaci-Salli

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