دورية أكاديمية
Human Huntington's Disease iPSC-Derived Cortical Neurons Display Altered Transcriptomics, Morphology, and Maturation.
| العنوان: | Human Huntington's Disease iPSC-Derived Cortical Neurons Display Altered Transcriptomics, Morphology, and Maturation. |
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| المؤلفون: | Mehta SR; The Board of Governor's Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA; Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA., Tom CM; The Board of Governor's Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA; Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA., Wang Y; Genomics Core Facility, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA., Bresee C; Biostatistics & Bioinformatics Research Center, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA., Rushton D; The Board of Governor's Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA; Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA., Mathkar PP; The Board of Governor's Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA; Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA., Tang J; Genomics Core Facility, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA., Mattis VB; The Board of Governor's Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA; Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA. Electronic address: virginiamattis@gmail.com. |
| المصدر: | Cell reports [Cell Rep] 2018 Oct 23; Vol. 25 (4), pp. 1081-1096.e6. |
| نوع المنشور: | Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
| اللغة: | English |
| بيانات الدورية: | Publisher: Cell Press Country of Publication: United States NLM ID: 101573691 Publication Model: Print Cited Medium: Internet ISSN: 2211-1247 (Electronic) NLM ISO Abbreviation: Cell Rep Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: [Cambridge, MA] : Cell Press, c 2012- |
| مواضيع طبية MeSH: | Cell Differentiation/*genetics , Cell Shape/*genetics , Cerebral Cortex/*pathology , Huntington Disease/*pathology , Induced Pluripotent Stem Cells/*pathology , Neurons/*pathology , Transcriptome/*genetics, Cells, Cultured ; Gene Regulatory Networks ; Humans ; Neurites/metabolism ; Neurons/metabolism ; Phenotype |
| مستخلص: | Huntington's disease (HD) is a neurodegenerative disease caused by an expanded CAG repeat in the Huntingtin (HTT) gene. Induced pluripotent stem cell (iPSC) models of HD provide an opportunity to study the mechanisms underlying disease pathology in disease-relevant patient tissues. Murine studies have demonstrated that HTT is intricately involved in corticogenesis. However, the effect of mutant Hungtintin (mtHTT) in human corticogenesis has not yet been thoroughly explored. This examination is critical, due to inherent differences in cortical development and timing between humans and mice. We therefore differentiated HD and non-diseased iPSCs into functional cortical neurons. While HD patient iPSCs can successfully differentiate toward a cortical fate in culture, the resulting neurons display altered transcriptomics, morphological and functional phenotypes indicative of altered corticogenesis in HD. (Copyright © 2018 The Author(s). Published by Elsevier Inc. All rights reserved.) |
| فهرسة مساهمة: | Keywords: Huntingtin; Huntington’s disease; cerebral cortex; corticogenesis; differentiation; iPSC |
| تواريخ الأحداث: | Date Created: 20181026 Date Completed: 20191114 Latest Revision: 20191114 |
| رمز التحديث: | 20231215 |
| DOI: | 10.1016/j.celrep.2018.09.076 |
| PMID: | 30355486 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 2211-1247 |
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| DOI: | 10.1016/j.celrep.2018.09.076 |