Characterization of Neurodevelopmental Abnormalities in iPSC-Derived Striatal Cultures from Patients with Huntington’s Disease (English)

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in Journal of Huntington's Disease ; 8 , 3 ; 257-269
Journal of Huntington's Disease
IOS Press , Amsterdam, The Netherlands; 2019

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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Table of contents – Volume 8, Issue 3

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Tables of content are generated automatically and are based on records of articles contained that are available in the TIB-Portal index. Due to missing records of articles, the volume display may be incomplete, even though the whole journal is available at TIB.

245
Glycation in Huntington’s Disease: A Possible Modifier and Target for Intervention
Brás, Inês Caldeira / König, Annekatrin / Outeiro, Tiago Fleming | 2019
257
Characterization of Neurodevelopmental Abnormalities in iPSC-Derived Striatal Cultures from Patients with Huntington’s Disease
Mathkar, Pranav P. / Suresh, Divya / Dunn, James / Tom, Colton M. / Mattis, Virginia B. | 2019
363
Huntington’s Disease Clinical Trials Corner: June 2019
Rodrigues, Filipe B. / Ferreira, Joaquim J. / Wild, Edward J. | 2019

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