ArticleDisease models & mechanisms2015
Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway.
Article in Disease models & mechanisms, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.
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Who cites it
36 citing papers in PubMed, 54 citations in OpenAlex.
- Brain organoid models of Huntington's disease shift the focus towards neurodevelopment.Disease models & mechanisms · 2025Article
- Oxidative Stress in Huntington's Disease.Biomolecules · 2025Review
- Proteomes of plasmodium knowlesi early and late ring-stage parasites and infected host erythrocytes.Journal of proteomics · 2024Article
- Protocol Optimization for Direct Reprogramming of Primary Human Fibroblast into Induced Striatal Neurons.International journal of molecular sciences · 2023Article
- Allele-specific quantitation of ATXN3 and HTT transcripts in polyQ disease models.BMC biology · 2023Article
- Disruption of Intracellular Calcium Homeostasis Leads to ERLIN2-Linked Hereditary Spastic Paraplegia in Patient-Derived Stem Cell Models.Human mutation · 2023Article
- SUPT4H1-edited stem cell therapy rescues neuronal dysfunction in a mouse model for Huntington's disease.NPJ Regenerative medicine · 2022Article
- Huntington's disease iPSC models-using human patient cells to understand the pathology caused by expanded CAG repeats.Faculty reviews · 2022Review
- Altered microRNA expression in animal models of Huntington's disease and potential therapeutic strategies.Neural regeneration research · 2021Review
- Multiomics landscape of the autosomal dominant osteopetrosis type II disease-specific induced pluripotent stem cells.Hereditas · 2021Article
- Review
- Patient-derived iPSC modeling of rare neurodevelopmental disorders: Molecular pathophysiology and prospective therapies.Neuroscience and biobehavioral reviews · 2021Review
- Neural In Vitro Models for Studying Substances Acting on the Central Nervous System.Handbook of experimental pharmacology · 2021Article
- Expanding the Arsenal Against Huntington's Disease-Herbal Drugs and Their Nanoformulations.Current neuropharmacology · 2021Review
- Juvenile Huntington's Disease and Other PolyQ Diseases, Update on Neurodevelopmental Character and Comparative Bioinformatic Review of Transcriptomic and Proteomic Data.Frontiers in cell and developmental biology · 2021Review
- Nicotinamide Adenine Dinucleotide Phosphate Oxidases Are Everywhere in Brain Disease, but Not in Huntington's Disease?Frontiers in aging neuroscience · 2021Review
- Neuroprotection: Targeting Multiple Pathways by Naturally Occurring Phytochemicals.Biomedicines · 2020Review
- Using induced pluripotent stem cell neuronal models to study neurodegenerative diseases.Biochimica et biophysica acta. Molecular basis of disease · 2020Review
- Recent Overview of the Use of iPSCs Huntington's Disease Modeling and Therapy.International journal of molecular sciences · 2020Review
- Mitochondrial and Redox Modifications in Huntington Disease Induced Pluripotent Stem Cells Rescued by CRISPR/Cas9 CAGs Targeting.Frontiers in cell and developmental biology · 2020Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
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Abstract
Huntington disease (HD) is a brain disorder characterized by the late onset of motor and cognitive symptoms, even though the neurons in the brain begin to suffer dysfunction and degeneration long before symptoms appear. There is currently no cure. Several molecular and developmental effects of HD have been identified using neural stem cells (NSCs) and differentiated cells, such as neurons and astrocytes. Still, little is known regarding the molecular pathogenesis of HD in pluripotent cells, such as embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs). Therefore, we examined putative signaling pathways and processes involved in HD pathogenesis in pluripotent cells. We tested naïve mouse HD YAC128 iPSCs and two types of human HD iPSC that were generated from HD and juvenile-HD patients. Surprisingly, we found that a number of changes affecting cellular processes in HD were also present in undifferentiated pluripotent HD iPSCs, including the dysregulation of the MAPK and Wnt signaling pathways and the dysregulation of the expression of genes related to oxidative stress, such as Sod1. Interestingly, a common protein interactor of the huntingtin protein and the proteins in the above pathways is p53, and the expression of p53 was dysregulated in HD YAC128 iPSCs and human HD iPSCs. In summary, our findings demonstrate that multiple molecular pathways that are characteristically dysregulated in HD are already altered in undifferentiated pluripotent cells and that the pathogenesis of HD might begin during the early stages of life.
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