Mechanisms of neuronal cell death in Huntington’s disease
- 1 July 2003
- journal article
- review article
- Published by S. Karger AG in Cytogenetic and Genome Research
- Vol. 100 (1-4) , 287-295
- https://doi.org/10.1159/000072864
Abstract
Huntington’s disease (HD) is a genetically dominant neurodegenerative condition caused by an unique mutation in the disease gene huntingtin. Although the Huntington protein (Htt) is ubiquitously expressed, expansion of the polyglutamine tract in Htt leads to the progressive loss of specific neuronal subpopulations in HD brains. In this article, we will summarize the current understanding on mechanisms of how mutant Htt can elicit cytotoxicity, as well as how the selective sets of neuronal cell death occur in HD brains.Keywords
This publication has 42 references indexed in Scilit:
- Identification of ter94, Drosophila VCP, as a modulator of polyglutamine-induced neurodegenerationCell Death & Differentiation, 2002
- Loss of normal huntingtin function: new developments in Huntington's disease researchTrends in Neurosciences, 2001
- Mutant Huntingtin Enhances Excitotoxic Cell DeathMolecular and Cellular Neuroscience, 2001
- Inhibiting Caspase Cleavage of Huntingtin Reduces Toxicity and Aggregate Formation in Neuronal and Nonneuronal CellsJournal of Biological Chemistry, 2000
- Protein ChainmailCell, 1998
- Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in BrainScience, 1997
- Exon 1 of the HD Gene with an Expanded CAG Repeat Is Sufficient to Cause a Progressive Neurological Phenotype in Transgenic MiceCell, 1996
- Amplification of EPSPs by axosomatic sodium channels in neocortical pyramidal neuronsNeuron, 1995
- Huntington's disease, energy, and excitotoxicityNeurobiology of Aging, 1994
- A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomesCell, 1993