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Adipose tissue-derived stem cells rescue Purkinje neurons and alleviate inflammatory responses in Niemann-Pick disease type C mice

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Abstract

Adult stem cells offer special therapeutic prospects because they can be isolated for autologous transplantation, expanded ex vivo, and differentiated into various cell types. We previously reported that bone marrow-derived mesenchymal stem cells improve neurological deficits in neurodegenerative disease animal models. However, the efficacy of adipose tissue-derived stem cells (ADSCs) transplantation in similar models remains unknown. Herein, we demonstrate that ADSCs, when transplanted into Niemann-Pick disease type C (NP-C) mouse cerebellum, elicit rescue of Purkinje neurons and restoration of motor coordination together with alleviation of inflammatory responses as verified by immunohistochemistry and real-time PCR using glial fibrillary acidic protein (GFAP), F4/80, IL-1β, IL-6, and TNF-α. Most importantly, ADSCs enhance electrically active Purkinje neurons with functional synaptic formation after transplantation in NP-C disease model mice. This report demonstrates for the first time that ADSCs can rescue imperiled Purkinje neurons and alleviate the inflammatory response in NP-C disease model mice, thereby signifying the therapeutic potential of ADSCs for neurodegenerative diseases.

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Authors and Affiliations

  1. Department of Physiology, Cell and Matrix Research Institute, BSEI, World Class University Program, School of Medicine, Kyungpook National University, Daegu, Korea

    Jae-sung Bae

  2. Department of Mental Health Sciences, Royal Free and University College Medical School, University College London, London, UK

    Janet E. Carter

  3. Department of Laboratory Animal Medicine, College of Veterinary Medicine, Cell and Matrix Research Institute, Kyungpook National University, Daegu, Korea

    Hee Kyung Jin

  4. School of Medicine, Kyungpook National University, 101 Dongindong 2Ga, Jung-Gu, Daegu, 700-422, South Korea

    Jae-sung Bae

  5. College of Veterinary Medicine, Kyungpook National University, 1370 Sankyuk-dong, Buk-gu, Daegu, 702-701, South Korea

    Hee Kyung Jin

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  1. Jae-sung Bae
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  2. Janet E. Carter
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Correspondence to Jae-sung Bae or Hee Kyung Jin.

Additional information

This work was supported by a grant (SC4170) from Stem Cell Research Center of the 21st Century Frontier Research Program funded by the Ministry of Science and Technology, Republic of Korea, to H.K.J, the Korea Research Foundation Grant funded by the Korea Government (MOEHRD) (KRF-2006-311-E00438) to H.K.J., and the Grant of the Korean Ministry of Education, Science and Technology (The Regional Core Research Program/Anti-aging and Well-being Research Center).

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Bae, Js., Carter, J.E. & Jin, H.K. Adipose tissue-derived stem cells rescue Purkinje neurons and alleviate inflammatory responses in Niemann-Pick disease type C mice. Cell Tissue Res 340, 357–369 (2010). https://doi.org/10.1007/s00441-010-0942-3

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  • DOI: https://doi.org/10.1007/s00441-010-0942-3

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