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Updated: May 27, 2026
07:08
Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
Published on: February 27, 2018
Neurodegenerative processes in Huntington's disease.
1Deutsches Zentrum für Neurodegenerative Erkrankungen, Bonn, Germany. daniele.bano@dzne.de
Cell Death & Disease
|November 11, 2011
Summary
Huntington's disease (HD) involves progressive neuron loss due to a mutant huntingtin protein. This review explores signaling pathways and age-dependent proteostasis loss contributing to neuronal degeneration and aggregate formation in HD.
Area of Science:
- Neuroscience
- Genetics
- Molecular Biology
Background:
- Huntington's disease (HD) is a severe neurodegenerative disorder.
- Characterized by progressive striatal neuron loss, leading to motor and cognitive deficits.
- Caused by expanded CAG trinucleotide repeats in the huntingtin gene.
Purpose of the Study:
- To review signaling pathways involved in neuronal degeneration in HD.
- To highlight the role of age-dependent proteostasis loss in HD pathogenesis.
- To discuss potential molecular targets for pharmacological interventions.
Main Methods:
- Review of in-vitro and in-vivo model investigations.
- Analysis of signaling pathways contributing to neurodegeneration.
- Examination of proteostasis mechanisms and aggregate formation.
Main Results:
- Mutant huntingtin protein expression drives toxic events.
- Age-dependent loss of proteostasis is critical for aggregate formation.
- Specific signaling pathways are implicated in neuronal death.
Conclusions:
- Understanding signaling pathways and proteostasis is key to HD research.
- Pharmacological targeting of identified molecular pathways offers therapeutic potential.
- Further research into HD pathogenesis can lead to effective treatments.
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