ArticleDisease models & mechanisms
Mouse models of Huntington disease: variations on a theme.
Article in Disease models & mechanisms. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 53 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
53 citing papers in PubMed, 100 citations in OpenAlex.
- Human-specific sequence features in HTT exon 1 promote toxic misprocessing via splicing factor SRSF7.Nature communications · 2026Article
- 3D-AI mouse behavior analysis system has the capability to detect abnormalities in R6/1 model mice with Huntington's disease during the pre-symptomatic phase.Frontiers in psychiatry · 2026Article
- Article
- Microglia and complement mediate early corticostriatal synapse loss and cognitive dysfunction in Huntington's disease.Nature medicine · 2023Article
- 3D Imaging of Striatal Transplants in a Small Animal Model of Huntington's Disease.Neurology international · 2023Article
- A Novel Huntington's Disease Assessment Platform to Support Future Drug Discovery and Development.International journal of molecular sciences · 2022Article
- Metabolism in Huntington's disease: a major contributor to pathology.Metabolic brain disease · 2022Review
- Article
- Converging evidence in support of omega-3 polyunsaturated fatty acids as a potential therapy for Huntington's disease symptoms.Reviews in the neurosciences · 2021Review
- Mitochondrial Abnormalities and Synaptic Damage in Huntington's Disease: a Focus on Defective Mitophagy and Mitochondria-Targeted Therapeutics.Molecular neurobiology · 2021Review
- C57BL/6 Background Attenuates mHTT Toxicity in the Striatum of YAC128 Mice.International journal of molecular sciences · 2021Article
- Spatiotemporal analysis of soluble aggregates and autophagy markers in the R6/2 mouse model.Scientific reports · 2021Article
- Huntington's disease mouse models: unraveling the pathology caused by CAG repeat expansion.Faculty reviews · 2021Review
- MicroRNAs in Huntington's Disease: Diagnostic Biomarkers or Therapeutic Agents?Frontiers in cellular neuroscience · 2021Review
- Modulating gene regulation to treat genetic disorders.Nature reviews. Drug discovery · 2020Review
- mGluR5 regulates REST/NRSF signaling through N-cadherin/β-catenin complex in Huntington's disease.Molecular brain · 2020Article
- CRISPR/Cas: a potential gene-editing tool in the nervous system.Cell regeneration (London, England) · 2020Review
- Abnormal Brain Development in Huntington' Disease Is Recapitulated in the zQ175 Knock-In Mouse Model.Cerebral cortex communications · 2020Article
- Ataxic Symptoms in Huntington's Disease Transgenic Mouse Model Are Alleviated by Chlorzoxazone.Frontiers in neuroscience · 2020Article
- CRISPR-Cas9-Mediated Genome Editing Increases Lifespan and Improves Motor Deficits in a Huntington's Disease Mouse Model.Molecular therapy. Nucleic acids · 2019Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
An accepted prerequisite for clinical trials of a compound in humans is the successful alleviation of the disease in animal models. For some diseases, however, successful translation of drug effects from mouse models to the bedside has been limited. One question is whether the current models accurately reproduce the human disease. Here, we examine the mouse models that are available for therapeutic testing in Huntington disease (HD), a late-onset neurodegenerative disorder for which there is no effective treatment. The current mouse models show different degrees of similarity to the human condition. Significant phenotypic differences are seen in mouse models that express either truncated or full-length human, or full-length mouse, mutant huntingtin (mHTT). These differences in phenotypic expression may be attributable to the influences of protein context, mouse strain and a difference in regulatory sequences between the mouse Htt and human HTT genes.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.