دورية أكاديمية
أعرض في EDS

Huntington's Disease Patient-Derived Astrocytes Display Electrophysiological Impairments and Reduced Neuronal Support.

التفاصيل البيبلوغرافية
العنوان: Huntington's Disease Patient-Derived Astrocytes Display Electrophysiological Impairments and Reduced Neuronal Support.
المؤلفون: 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.; Divisions of Biomedicine and Neuroscience, School of Biosciences, Cardiff University, Cardiff, United Kingdom., Tom CM; Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United States., Allen ND; Divisions of Biomedicine and Neuroscience, School of Biosciences, Cardiff University, Cardiff, United Kingdom., Kemp PJ; Divisions of Biomedicine and Neuroscience, School of Biosciences, Cardiff University, Cardiff, United Kingdom., Svendsen CN; Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United States., Mattis VB; Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA, United States.
المصدر: Frontiers in neuroscience [Front Neurosci] 2019 Jun 28; Vol. 13, pp. 669. Date of Electronic Publication: 2019 Jun 28 (Print Publication: 2019).
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Frontiers Research Foundation Country of Publication: Switzerland NLM ID: 101478481 Publication Model: eCollection Cited Medium: Print ISSN: 1662-4548 (Print) Linking ISSN: 1662453X NLM ISO Abbreviation: Front Neurosci Subsets: PubMed not MEDLINE
أسماء مطبوعة: Original Publication: Lausanne : Frontiers Research Foundation
مستخلص: 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 Ca 2+ 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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معلومات مُعتمدة: MR/L023784/1 United Kingdom MRC_ Medical Research Council; MR/L023784/2 United Kingdom MRC_ Medical Research Council
فهرسة مساهمة: Keywords: HD; HTT; Huntington’s disease; astrocyte; co-culture; iPSC; neurodegeneration; neuron
تواريخ الأحداث: Date Created: 20190719 Latest Revision: 20210110
رمز التحديث: 20221213
مُعرف محوري في PubMed: PMC6610155
DOI: 10.3389/fnins.2019.00669
PMID: 31316341
قاعدة البيانات: MEDLINE
الوصف
تدمد:1662-4548
DOI:10.3389/fnins.2019.00669