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Huntington's Disease Patient-Derived Astrocytes Display Electrophysiological Impairments and Reduced Neuronal Support.
Garcia, Veronica J; Rushton, David J; Tom, Colton M; Allen, Nicholas D; Kemp, Paul J; Svendsen, Clive N; Mattis, Virginia B.
Afiliación
  • Garcia VJ; Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United States.
  • Rushton DJ; Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United States.
  • Tom CM; Divisions of Biomedicine and Neuroscience, School of Biosciences, Cardiff University, Cardiff, United Kingdom.
  • Allen ND; Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United States.
  • Kemp PJ; Divisions of Biomedicine and Neuroscience, School of Biosciences, Cardiff University, Cardiff, United Kingdom.
  • Svendsen CN; Divisions of Biomedicine and Neuroscience, School of Biosciences, Cardiff University, Cardiff, United Kingdom.
  • Mattis VB; Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United States.
Front Neurosci ; 13: 669, 2019.
Article en En | MEDLINE | ID: mdl-31316341
In Huntington's disease (HD), while the ubiquitously expressed mutant Huntingtin (mtHTT) protein primarily compromises striatal and cortical neurons, glia also undergo disease-contributing alterations. Existing HD models using human induced pluripotent stem cells (iPSCs) have not extensively characterized the role of mtHTT in patient-derived astrocytes. Here physiologically mature astrocytes are generated from HD patient iPSCs. These human astrocytes exhibit hallmark HD phenotypes that occur in mouse models, including impaired inward rectifying K+ currents, lengthened spontaneous Ca2+ waves and reduced cell membrane capacitance. HD astrocytes in co-culture provided reduced support for the maturation of iPSC-derived neurons. In addition, neurons exposed to chronic glutamate stimulation are not protected by HD astrocytes. This iPSC-based HD model demonstrates the critical effects of mtHTT on human astrocytes, which not only broadens the understanding of disease susceptibility beyond cortical and striatal neurons but also increases potential drug targets.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Front Neurosci Año: 2019 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Suiza
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Front Neurosci Año: 2019 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Suiza