Mitochondrial and metabolic-based protective strategies in Huntington’s disease: the case of creatine and coenzyme Q
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Luana Naia
Abstract
Huntington’s disease (HD) is a neurodegenerative genetic disorder caused by an expansion of CAG repeats in the HD gene encoding for huntingtin (Htt), resulting in progressive death of striatal neurons, with clinical symptoms of chorea, dementia and dramatic weight loss. Metabolic and mitochondrial dysfunction caused by the expanded polyglutamine sequence have been described along with other mechanisms of neurodegeneration previously described in human tissues and animal models of HD. In this review, we focus on mitochondrial and metabolic disturbances affecting both the central nervous system and peripheral cells, including mitochondrial DNA damage, mitochondrial complexes defects, loss of calcium homeostasis and transcriptional deregulation. Glucose abnormalities have also been described in peripheral tissues of HD patients and in HD animal and cellular models. Moreover, there are no effective neuroprotective treatments available in HD. Thus, we briefly discuss the role of creatine and coenzyme Q10 that target mitochondrial dysfunction and impaired bioenergetics and have been previously used in HD clinical trials.
©2012 by Walter de Gruyter Berlin Boston
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- Mitochondrial and metabolic-based protective strategies in Huntington’s disease: the case of creatine and coenzyme Q
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- Theta phase precession beyond the hippocampus
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- Head models and dynamic causal modeling of subcortical activity using magnetoencephalographic/electroencephalographic data
- The role of glutamatergic inputs onto parvalbumin-positive interneurons: relevance for schizophrenia
- Stress-linked cortisol concentrations in hair: what we know and what we need to know
- Prelims
- Prelims
Articles in the same Issue
- GSK3: a key target for the development of novel treatments for type 2 diabetes mellitus and Alzheimer disease
- Mitochondrial and metabolic-based protective strategies in Huntington’s disease: the case of creatine and coenzyme Q
- At a PI3K crossroads: lessons from flies and rodents
- Theta phase precession beyond the hippocampus
- The role of Ca2+-stimulated adenylyl cyclases in bidirectional synaptic plasticity and brain function
- Endoplasmic reticulum stress and prion diseases
- Head models and dynamic causal modeling of subcortical activity using magnetoencephalographic/electroencephalographic data
- The role of glutamatergic inputs onto parvalbumin-positive interneurons: relevance for schizophrenia
- Stress-linked cortisol concentrations in hair: what we know and what we need to know
- Prelims
- Prelims
