Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

iPS Cell Differentiation01:22

iPS Cell Differentiation

3.0K
The ability of induced pluripotent stem cells or iPSCs to differentiate into most body cell types has stimulated repair and regenerative medicine research over the past few decades. iPSC-derived blood cells, hepatocytes, beta islet cells, cardiomyocytes, neurons, and other cell types can repair injuries or regenerate damaged tissue in diseases such as diabetes and neurodegenerative disorders.
3.0K
EPS and iPS Cells in Disease Research01:21

EPS and iPS Cells in Disease Research

3.2K
Embryonic and induced pluripotent stem cells are excellent models for disease research because of their ability to self-renew and differentiate into most cell types. Somatic cells from a patient are isolated and reprogrammed into induced pluripotent stem cells or iPSCs. These iPSCs are later differentiated into the desired cell type, which mirrors the diseased cell of the patient. In this way, disease models have been created for investigating diseases such as Down syndrome, type I diabetes,...
3.2K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

iPSC Neurodegenerative Disease Initiative isogenic CAG repeat iPSC line for Huntington's disease.

bioRxiv : the preprint server for biology·2026
Same author

An in vivo platform to jointly monitor cellular and metabolic responses to chemotherapy.

bioRxiv : the preprint server for biology·2026
Same author

Single-cell RNA profiling of oligodendroglial lineage cells derived from iPSCs carrying Parkinson's disease-relevant LRRK2-G2019S mutation.

iScience·2026
Same author

Systematic evaluation of single-cell multimodal data integration enhances cell type resolution and discovery of clinically relevant states in complex tissues.

Genome biology·2026
Same author

Wild-type <i>C9orf72</i> expression is a genetic modifier of C9-ALS survival.

medRxiv : the preprint server for health sciences·2026
Same author

Integration of multiomic and multi-phenotypic data identifies biological pathways associated with physical fitness.

Communications biology·2026

Related Experiment Video

Updated: Dec 27, 2025

A Guide to Generating and Using hiPSC Derived NPCs for the Study of Neurological Diseases
09:30

A Guide to Generating and Using hiPSC Derived NPCs for the Study of Neurological Diseases

Published on: February 21, 2015

18.9K

Aberrant Development Corrected in Adult-Onset Huntington's Disease iPSC-Derived Neuronal Cultures via WNT Signaling

Charlene Smith-Geater1, Sarah J Hernandez2, Ryan G Lim3

  • 1Department of Psychiatry and Human Behavior, University of California Irvine, Irvine, CA 92697, USA.

Stem Cell Reports
|February 29, 2020
PubMed
Summary

Huntington's disease (HD) involves abnormal brain development. Researchers found persistent neural stem cells (NSCs) in HD patient cells, suggesting a new therapeutic target for neurodevelopmental disorders.

Keywords:
Huntington's diseaseWNT signalingadult-onset HDcell cycledevelopmentinduced pluripotent stem cellsmedium spiny neuronsneural stem cellssingle-cell RNA-seq

More Related Videos

Establishment of an Electrophysiological Platform for Modeling ALS with Regionally-Specific Human Pluripotent Stem Cell-Derived Astrocytes and Neurons
11:52

Establishment of an Electrophysiological Platform for Modeling ALS with Regionally-Specific Human Pluripotent Stem Cell-Derived Astrocytes and Neurons

Published on: August 26, 2021

2.6K
2D and 3D Human Induced Pluripotent Stem Cell-Based Models to Dissect Primary Cilium Involvement during Neocortical Development
14:19

2D and 3D Human Induced Pluripotent Stem Cell-Based Models to Dissect Primary Cilium Involvement during Neocortical Development

Published on: March 25, 2022

4.3K

Related Experiment Videos

Last Updated: Dec 27, 2025

A Guide to Generating and Using hiPSC Derived NPCs for the Study of Neurological Diseases
09:30

A Guide to Generating and Using hiPSC Derived NPCs for the Study of Neurological Diseases

Published on: February 21, 2015

18.9K
Establishment of an Electrophysiological Platform for Modeling ALS with Regionally-Specific Human Pluripotent Stem Cell-Derived Astrocytes and Neurons
11:52

Establishment of an Electrophysiological Platform for Modeling ALS with Regionally-Specific Human Pluripotent Stem Cell-Derived Astrocytes and Neurons

Published on: August 26, 2021

2.6K
2D and 3D Human Induced Pluripotent Stem Cell-Based Models to Dissect Primary Cilium Involvement during Neocortical Development
14:19

2D and 3D Human Induced Pluripotent Stem Cell-Based Models to Dissect Primary Cilium Involvement during Neocortical Development

Published on: March 25, 2022

4.3K

Area of Science:

  • Neuroscience
  • Stem Cell Biology
  • Genetics

Background:

  • Aberrant neuronal development and persistent mitotic cells are linked to neurological disorders like Huntington's disease (HD).
  • The precise mechanisms driving these pathologies in HD remain largely unknown.
  • Medium spiny neurons are critically affected in HD, making them a key focus for research.

Purpose of the Study:

  • To investigate the mechanisms underlying aberrant neurodevelopment in Huntington's disease.
  • To identify specific cellular populations contributing to HD pathology during neuronal differentiation.
  • To explore potential therapeutic strategies targeting identified mechanisms.

Main Methods:

  • Utilized a modified protocol to differentiate induced pluripotent stem cells (iPSCs) from HD patients and controls into neuronal cultures.
  • Enriched neuronal cultures for medium spiny neurons, the cell type most impacted in HD.
  • Conducted single-cell and bulk transcriptomic and epigenomic analyses to profile cellular changes.

Main Results:

  • Identified a persistent cyclin D1-positive neural stem cell (NSC) population exclusively in adult-onset HD iPSC-derived neuronal cultures.
  • Demonstrated that WNT pathway inhibition effectively eliminates this aberrant NSC population.
  • Showed that WNT inhibition preserves neuronal cells during the differentiation process.

Conclusions:

  • Aberrant neurodevelopment in adult-onset HD may involve a persistent NSC population.
  • This persistent NSC population could be a key mechanism contributing to HD pathology.
  • WNT pathway inhibition presents a potential therapeutic approach to correct aberrant neurodevelopment in HD striatal neurons.