ArticleFrontiers in cellular neuroscience2013
Characterization of forebrain neurons derived from late-onset Huntington's disease human embryonic stem cell lines.
Article in Frontiers in cellular neuroscience, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 36 citations in OpenAlex.
- Review
- Do Post-Translational Modifications Influence Protein Aggregation in Neurodegenerative Diseases: A Systematic Review.Brain sciences · 2020Review
- Energy Metabolism and Mitochondrial Superoxide Anion Production in Pre-symptomatic Striatal Neurons Derived from Human-Induced Pluripotent Stem Cells Expressing Mutant Huntingtin.Molecular neurobiology · 2020Article
- Modeling Polyglutamine Expansion Diseases with Induced Pluripotent Stem Cells.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2019Review
- Huntington Disease as a Neurodevelopmental Disorder and Early Signs of the Disease in Stem Cells.Molecular neurobiology · 2018Review
- Efficiently Specified Ventral Midbrain Dopamine Neurons from Human Pluripotent Stem Cells Under Xeno-Free Conditions Restore Motor Deficits in Parkinsonian Rodents.Stem cells translational medicine · 2017Article
- Induced Pluripotent Stem Cell-Derived Neural Stem Cell Transplantations Reduced Behavioral Deficits and Ameliorated Neuropathological Changes in YAC128 Mouse Model of Huntington's Disease.Frontiers in neuroscience · 2017Article
- GAPTrap: A Simple Expression System for Pluripotent Stem Cells and Their Derivatives.Stem cell reports · 2016Article
- Induced Pluripotent Stem Cells in Huntington's Disease Research: Progress and Opportunity.Journal of Huntington's disease · 2016Review
- iPSC-based drug screening for Huntington's disease.Brain research · 2016Review
- Importance of being Nernst: Synaptic activity and functional relevance in stem cell-derived neurons.World journal of stem cells · 2015Review
- Epigenetic changes in the developing brain: Effects on behavior.Proceedings of the National Academy of Sciences of the United States of America · 2015Article
- Advances in reprogramming-based study of neurologic disorders.Stem cells and development · 2015Review
- Article
- Investigating human disease using stem cell models.Nature reviews. Genetics · 2014Review
- Developing stem cell-based therapies for neural repair.Frontiers in cellular neuroscience · 2013Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Huntington's disease (HD) is an incurable neurodegenerative disorder caused by a CAG repeat expansion in exon 1 of the Huntingtin (HTT) gene. Recently, induced pluripotent stem cell (iPSC) lines carrying atypical and aggressive (CAG60+) HD variants have been generated and exhibit disparate molecular pathologies. Here we investigate two human embryonic stem cell (hESC) lines carrying CAG37 and CAG51 typical late-onset repeat expansions in comparison to wildtype control lines during undifferentiated states and throughout forebrain neuronal differentiation. Pluripotent HD lines demonstrate growth, viability, pluripotent gene expression, mitochondrial activity and forebrain specification that is indistinguishable from control lines. Expression profiles of crucial genes known to be dysregulated in HD remain unperturbed in the presence of mutant protein and throughout differentiation; however, elevated glutamate-evoked responses were observed in HD CAG51 neurons. These findings suggest typical late-onset HD mutations do not alter pluripotent parameters or the capacity to generate forebrain neurons, but that such progeny may recapitulate hallmarks observed in established HD model systems. Such HD models will help further our understanding of the cascade of pathological events leading to disease onset and progression, while simultaneously facilitating the identification of candidate HD therapeutics.
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