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Patient-Specific Induced Pluripotent Stem Cell Models pp 309–321Cite as

Modeling Axonal Phenotypes with Human Pluripotent Stem Cells

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Part of the book series: Methods in Molecular Biology ((MIMB,volume 1353))

Abstract

Impaired axonal development and degeneration are implicated in many debilitating disorders, such as hereditary spastic paraplegia (HSP), amyotrophic lateral sclerosis (ALS), and periphery neuropathy. Human pluripotent stem cells (hPSCs) have provided researchers with an excellent resource for modeling human neuropathologic processes including axonal defects in vitro. There are a number of steps that are crucial when developing an hPSC-based model of a human disease, including generating induced pluripotent stem cells (iPSCs), differentiating those cells to affected cell types, and identifying disease-relevant phenotypes. Here, we describe these steps in detail, focusing on the neurodegenerative disorder HSP.

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Acknowledgments

This work was supported by the Spastic Paraplegia Foundation and a Connecticut Stem Cell Research Grant (11SCB24).

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Authors and Affiliations

  1. Department of Neuroscience, University of Connecticut Health Center, 263 Farmington Avenue, Farmington, CT, 06030, USA

    Kyle R. Denton, Chong-Chong Xu & Xue-Jun Li

  2. The Stem Cell Institute, University of Connecticut Health Center, Farmington, CT, 06032, USA

    Xue-Jun Li

Authors
  1. Kyle R. Denton
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  2. Chong-Chong Xu
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  3. Xue-Jun Li
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Corresponding author

Correspondence to Xue-Jun Li .

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Editors and Affiliations

  1. Lunenfeld-Tanenbaum Research Institute, Toronto, Ontario, Canada

    Andras Nagy

  2. Ottawa Hospital Research Institute, Ottawa, Ontario, Canada

    Kursad Turksen

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© 2014 Springer Science+Business Media New York

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Denton, K.R., Xu, CC., Li, XJ. (2014). Modeling Axonal Phenotypes with Human Pluripotent Stem Cells. In: Nagy, A., Turksen, K. (eds) Patient-Specific Induced Pluripotent Stem Cell Models. Methods in Molecular Biology, vol 1353. Humana Press, New York, NY. https://doi.org/10.1007/7651_2014_167

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  • DOI: https://doi.org/10.1007/7651_2014_167

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  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4939-3033-3

  • Online ISBN: 978-1-4939-3034-0

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