دورية أكاديمية
Characterization of Neurodevelopmental Abnormalities in iPSC-Derived Striatal Cultures from Patients with Huntington's Disease.
| العنوان: | Characterization of Neurodevelopmental Abnormalities in iPSC-Derived Striatal Cultures from Patients with Huntington's Disease. |
|---|---|
| المؤلفون: | Mathkar PP; The Board of Governors Regenerative Medicine Institute and Department of Biomedical Sciences; Cedars-Sinai Medical Center, Los Angeles, CA, USA., Suresh D; The Board of Governors Regenerative Medicine Institute and Department of Biomedical Sciences; Cedars-Sinai Medical Center, Los Angeles, CA, USA., Dunn J; The Board of Governors Regenerative Medicine Institute and Department of Biomedical Sciences; Cedars-Sinai Medical Center, Los Angeles, CA, USA., Tom CM; The Board of Governors Regenerative Medicine Institute and Department of Biomedical Sciences; Cedars-Sinai Medical Center, Los Angeles, CA, USA., Mattis VB; The Board of Governors Regenerative Medicine Institute and Department of Biomedical Sciences; Cedars-Sinai Medical Center, Los Angeles, CA, USA. |
| المصدر: | Journal of Huntington's disease [J Huntingtons Dis] 2019; Vol. 8 (3), pp. 257-269. |
| نوع المنشور: | Journal Article; Research Support, Non-U.S. Gov't |
| اللغة: | English |
| بيانات الدورية: | Publisher: IOS Press Country of Publication: Netherlands NLM ID: 101589965 Publication Model: Print Cited Medium: Internet ISSN: 1879-6400 (Electronic) Linking ISSN: 18796397 NLM ISO Abbreviation: J Huntingtons Dis Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Amsterdam, The Netherlands : IOS Press |
| مواضيع طبية MeSH: | Corpus Striatum/*growth & development , Huntington Disease/*physiopathology , Induced Pluripotent Stem Cells/*physiology , Neurons/*physiology, Cell Differentiation ; Cells, Cultured ; Corpus Striatum/physiopathology ; Humans ; Induced Pluripotent Stem Cells/metabolism ; Nestin/metabolism ; Receptors, Notch/metabolism |
| مستخلص: | Background: Huntington's disease (HD) is an inherited neurodegenerative disease and is characterized by atrophy of certain regions of the brain in a progressive manner. HD patients experience behavioral changes and uncontrolled movements which can be primarily attributed to the atrophy of striatal neurons. Previous publications describe the models of the HD striatum using induced pluripotent stem cells (iPSCs) derived from HD patients with a juvenile onset (JHD). In this model, the JHD iPSC-derived striatal cultures had altered neurodevelopment and contained a high number of nestin expressing progenitor cells at 42 days of differentiation. Objective: To further characterize the altered neurodevelopmental phenotype and evaluate potential phenotypic reversal. Methods: Differentiation of human iPSCs towards striatal fate and characterization by means of immunocytochemistry and stereological quantification. Results: Here this study demonstrates a distinct delay in the differentiation of the JHD neural progenitor population. However, reduction of the JHD aberrant progenitor populations can be accomplished either by targeting the canonical Notch signaling pathway or by treatment with HTT antisense oligonucleotides (ASOs). Conclusions: In summary, this data is postulated to reflect a potential overall developmental delay in JHD. |
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| فهرسة مساهمة: | Keywords: Huntington’s disease; huntingtin; iPSC; nestin; neurodegenerative disease |
| المشرفين على المادة: | 0 (NES protein, human) 0 (Nestin) 0 (Receptors, Notch) |
| تواريخ الأحداث: | Date Created: 20190806 Date Completed: 20200708 Latest Revision: 20240721 |
| رمز التحديث: | 20240721 |
| مُعرف محوري في PubMed: | PMC6839479 |
| DOI: | 10.3233/JHD-180333 |
| PMID: | 31381521 |
| قاعدة البيانات: | MEDLINE |
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