ArticleMolecular neurobiology2001
Modeling Huntington's disease in cells, flies, and mice.
Article in Molecular neurobiology, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 79 citations in OpenAlex.
- Bioinformatics analysis of Ras homologue enriched in the striatum, a potential target for Huntington's disease therapy.International journal of molecular medicine · 2019Article
- Studying polyglutamine diseases in Drosophila.Experimental neurology · 2015Review
- The Incremental Induction of Neuroprotective Properties by Multiple Therapeutic Strategies for Primary and Secondary Neural Injury.International journal of molecular sciences · 2015Review
- Novel BAC Mouse Model of Huntington's Disease with 225 CAG Repeats Exhibits an Early Widespread and Stable Degenerative Phenotype.Journal of Huntington's disease · 2015Article
- Article
- Evidence for dynamic and multiple roles for huntingtin in Ciona intestinalis.Invertebrate neuroscience : IN · 2013Article
- An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity.Cell death & disease · 2012Review
- Magnetic resonance imaging of Huntington's disease: preparing for clinical trials.Neuroscience · 2009Review
- Mutant huntingtin and mitochondrial dysfunction.Trends in neurosciences · 2008Review
- Characterization of gene expression regulated by human OTK18 using Drosophila melanogaster as a model system for innate immunity.Journal of genetics · 2008Article
- High throughput screening for neurodegeneration and complex disease phenotypes.Combinatorial chemistry & high throughput screening · 2008Review
- The cellular prion protein (PrP(C)): its physiological function and role in disease.Biochimica et biophysica acta · 2007Review
- Huntingtin gene evolution in Chordata and its peculiar features in the ascidian Ciona genus.BMC genomics · 2006Article
- Apoptotic cascades as possible targets for inhibiting cell death in Huntington's disease.Journal of neurology · 2006Review
- Development of an ischemic tolerance model in a PC12 cell line.Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism · 2005Article
- Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2.Proceedings of the National Academy of Sciences of the United States of America · 2003Article
- Huntingtin in health and disease.The Journal of clinical investigation · 2003Review
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
A milestone in Huntington's disease (HD) research is represented by the identification of the causative gene. With the genetics at hand, a series of transgenic cellular and animal models has been developed, which has greatly contributed to understanding of HD. All these models are described in this review, and are compared to each other, along with the information they have generated. Although the mechanism by which progressive loss of striatal neurons occurs in HD remains uncertain, hypotheses on mutant huntingtin toxicity involve impaired vescicular trafficking, transcriptional dysregulation, and/or activation of apoptotic pathways. The development of inducible HD mice has shown that neurodegeneration in HD may be at least partially blocked. Although traditionally considered a "gain-of-function" disease, the recent finding that normal huntingtin has an important role in neuronal survival suggests that loss of function of the normal protein might contribute to HD as well, also discloseing new perspectives on the therapeutical approach to the pathology.
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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.