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Published on: June 30, 2023
Huntington's disease and mitochondrial alterations: emphasis on experimental models
Verónica Pérez-De la Cruz1, Paul Carrillo-Mora, Abel Santamaría
1Laboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurología y Neurocirugía, Mexico City, 14269, Mexico.
Journal of Bioenergetics and Biomembranes
|June 5, 2010
Summary
Huntington's disease (HD) involves brain cell degeneration, particularly impacting mitochondria. Research highlights mitochondrial dysfunction as a key factor in HD, guiding the development of targeted therapies.
Area of Science:
- Neuroscience
- Genetics
- Cell Biology
Background:
- Huntington's disease (HD) is an inherited neurological disorder characterized by basal ganglia degeneration, chorea, and dementia.
- Mitochondrial deterioration, affecting both structure and function, is a significant factor in HD-related neurodegeneration.
Purpose of the Study:
- To review experimental studies investigating the role of mitochondria in Huntington's disease pathogenesis.
- To support the hypothesis that mitochondrial alterations are central to HD.
Main Methods:
- Review of experimental models in animals and cell preparations.
- Analysis of studies focusing on mechanistic alterations in HD mitochondria.
Main Results:
- Experimental models have elucidated mechanistic alterations in mitochondria relevant to HD.
- Evidence suggests mitochondrial dysfunction is a core component of HD pathology.
Conclusions:
- Mitochondria play a critical role in the pathogenesis of Huntington's disease.
- Targeting mitochondrial alterations presents a promising therapeutic strategy for HD.
