دورية أكاديمية
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The difficulty to model Huntington's disease in vitro using striatal medium spiny neurons differentiated from human induced pluripotent stem cells.

التفاصيل البيبلوغرافية
العنوان: The difficulty to model Huntington's disease in vitro using striatal medium spiny neurons differentiated from human induced pluripotent stem cells.
المؤلفون: Le Cann K; Institute of Physiology, RWTH Aachen University, Pauwelsstrasse 30, 52074, Aachen, Germany., Foerster A; Institute of Physiology, RWTH Aachen University, Pauwelsstrasse 30, 52074, Aachen, Germany., Rösseler C; Institute of Physiology, RWTH Aachen University, Pauwelsstrasse 30, 52074, Aachen, Germany., Erickson A; Institute of Physiology, RWTH Aachen University, Pauwelsstrasse 30, 52074, Aachen, Germany., Hautvast P; Institute of Physiology, RWTH Aachen University, Pauwelsstrasse 30, 52074, Aachen, Germany., Giesselmann S; Intitute of Human Genetic, RWTH Aachen University, 52074, Aachen, Germany., Pensold D; Institute of Biology II, Division of Functional Epigenetics in the Animal Model, RWTH Aachen University, 52074, Aachen, Germany., Kurth I; Intitute of Human Genetic, RWTH Aachen University, 52074, Aachen, Germany., Rothermel M; Institute Für Biology II, Department Chemosensation, AG Neuromodulation, 52074, Aachen, Germany., Mattis VB; Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA.; Fujifilm Cellular Dynamics, Madison, WI, 53711, USA., Zimmer-Bensch G; Institute of Biology II, Division of Functional Epigenetics in the Animal Model, RWTH Aachen University, 52074, Aachen, Germany., von Hörsten S; Intitute of Virology, Clinical and Molecular Virology, Animal Center of Preclinical Experiments (PETZ), 91054, Erlangen, Germany., Denecke B; Aachen - RWTH Aachen, 52074, Aachen, Germany., Clarner T; Intitute for Neuroanatomy, MIT 1, 52074, Aachen, Germany., Meents J; Institute of Physiology, RWTH Aachen University, Pauwelsstrasse 30, 52074, Aachen, Germany. jmeents@multichannelsystems.com.; Multi Channel Systems MCS GmbH, Aspenhaustrasse 21, 72770, Reutlingen, Germany. jmeents@multichannelsystems.com., Lampert A; Institute of Physiology, RWTH Aachen University, Pauwelsstrasse 30, 52074, Aachen, Germany. alampert@ukaachen.de.
المصدر: Scientific reports [Sci Rep] 2021 Mar 25; Vol. 11 (1), pp. 6934. Date of Electronic Publication: 2021 Mar 25.
نوع المنشور: Comparative Study; Evaluation Study; Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : Nature Publishing Group, copyright 2011-
مواضيع طبية MeSH: Cell Culture Techniques* , Cell Differentiation* , Huntington Disease*, Neurons/*physiology, Action Potentials ; Animals ; Calcium/metabolism ; Case-Control Studies ; Cell Line ; Humans ; Induced Pluripotent Stem Cells ; Mice, Inbred C57BL ; Voltage-Gated Sodium Channel beta-4 Subunit/metabolism ; gamma-Aminobutyric Acid/metabolism ; Mice
مستخلص: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by an expanded polyglutamine repeat in the huntingtin gene. The neuropathology of HD is characterized by the decline of a specific neuronal population within the brain, the striatal medium spiny neurons (MSNs). The origins of this extreme vulnerability remain unknown. Human induced pluripotent stem cell (hiPS cell)-derived MSNs represent a powerful tool to study this genetic disease. However, the differentiation protocols published so far show a high heterogeneity of neuronal populations in vitro. Here, we compared two previously published protocols to obtain hiPS cell-derived striatal neurons from both healthy donors and HD patients. Patch-clamp experiments, immunostaining and RT-qPCR were performed to characterize the neurons in culture. While the neurons were mature enough to fire action potentials, a majority failed to express markers typical for MSNs. Voltage-clamp experiments on voltage-gated sodium (Nav) channels revealed a large variability between the two differentiation protocols. Action potential analysis did not reveal changes induced by the HD mutation. This study attempts to demonstrate the current challenges in reproducing data of previously published differentiation protocols and in generating hiPS cell-derived striatal MSNs to model a genetic neurodegenerative disorder in vitro.
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المشرفين على المادة: 0 (Voltage-Gated Sodium Channel beta-4 Subunit)
56-12-2 (gamma-Aminobutyric Acid)
SY7Q814VUP (Calcium)
تواريخ الأحداث: Date Created: 20210326 Date Completed: 20211027 Latest Revision: 20240226
رمز التحديث: 20240226
مُعرف محوري في PubMed: PMC7994641
DOI: 10.1038/s41598-021-85656-x
PMID: 33767215
قاعدة البيانات: MEDLINE
الوصف
تدمد:2045-2322
DOI:10.1038/s41598-021-85656-x