ArticleCellular and molecular neurobiology2011
Modeling pathogenesis of Huntington's disease with inducible neuroprogenitor cells.
Article in Cellular and molecular neurobiology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 20 citations in OpenAlex.
- Heat Shock-Induced Extracellular Vesicles Derived from Neural Stem Cells Confer Marked Neuroprotection Against Oxidative Stress and Amyloid-β-Caused Neurotoxicity.Molecular neurobiology · 2022Article
- Conditioned medium from BV2 microglial cells having polyleucine specifically alters startle response in mice.Scientific reports · 2022Article
- Exogenous polyserine and polyleucine are toxic to recipient cells.Scientific reports · 2022Article
- Toxicity of internalized polyalanine to cells depends on aggregation.Scientific reports · 2021Article
- FOXOs modulate proteasome activity in human-induced pluripotent stem cells of Huntington's disease and their derived neural cells.Human molecular genetics · 2017Article
- Adult neural progenitor cells from Huntington's disease mouse brain exhibit increased proliferation and migration due to enhanced calcium and ROS signals.Cell proliferation · 2015Article
- Sulforaphane enhances proteasomal and autophagic activities in mice and is a potential therapeutic reagent for Huntington's disease.Journal of neurochemistry · 2014Article
- Article
- An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity.Cell death & disease · 2012Review
- Pluripotent stem cells models for Huntington's disease: prospects and challenges.Journal of genetics and genomics = Yi chuan xue bao · 2012Review
- Multiple phenotypes in Huntington disease mouse neural stem cells.Molecular and cellular neurosciences · 2012Article
- Prothymosin-α interacts with mutant huntingtin and suppresses its cytotoxicity in cell culture.The Journal of biological chemistry · 2012Article
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
Abstract
Huntington's disease (HD) is caused by an abnormal expansion of CAG trinucleotide repeats encoding polyglutamine (polyQ) in the first exon of the huntingtin (htt) gene. Despite considerable efforts, the pathogenesis of HD remains largely unclear due to a paucity of models that can reliably reproduce the pathological characteristics of HD. Here, we report a neuronal cell model of HD using the previously established tetracycline regulated rat neuroprogenitor cell line, HC2S2. Stable expression of enhanced green fluorescence protein tagged htt exon 1 (referred to as 28Q and 74Q, respectively) in the HC2S2 cells did not affect rapid neuronal differentiation. However, compared to the cells expressing wild type htt, the cell line expressing mutant htt showed an increase in time-dependent cell death and neuritic degeneration, and displayed increased vulnerability to oxidative stress. Increased protein aggregation during the process of neuronal aging or when the cells were exposed to oxidative stress reagents was detected in the cell line expressing 74Q but not in its counterpart. These results suggest that the neuroprogenitor cell lines mimic the major neuropathological characteristics of HD and may provide a useful tool for studying the neuropathogenesis of HD and for high throughput screening of therapeutic compounds.
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