Towards a comprehensive understanding of the contributions of mitochondrial dysfunction and oxidative stress in the pathogenesis and pathophysiology of Huntington’s disease.

Towards a comprehensive understanding of the contributions of mitochondrial dysfunction and oxidative stress in the pathogenesis and pathophysiology of Huntington’s disease.

Publication date: Jul 15, 2019

Huntington’s disease (HD) is a rare autosomal dominant disorder affecting the corticostriatal area of the brain. HD is driven by elongated cytosine-adenine-guanine (CAG) repeat (36 repeats or more) on the short arm of chromosome 4p16.3 in the Huntingtin gene (HTTg) which encode the huntingtin protein (HTT). Although the polyglutamine expansion within HTT is the causative factor in the pathogenesis of HD, the underlying mechanisms that provoke this expansion and the resulting neurodegeneration and clinical symptoms are not fully understood. In this paper, the critical role played by mitochondria dysfunction and oxidative stress in HTT expansion, HD progression, and clinical symptoms are elucidated. Their interactions with the key factors in the disease, as well as treatment strategies, are discussed.

Tobore, T.O. Towards a comprehensive understanding of the contributions of mitochondrial dysfunction and oxidative stress in the pathogenesis and pathophysiology of Huntington’s disease. 06578. 2019 J Neurosci Res.

Concepts Keywords
Adenine Huntingtin-interacting protein 1
Autosomal Dominant Oxidative stress
Brain HTT
Causative Trinucleotide repeat disorder
Chromosome Huntingtin
Corticostriatal Neurodegeneration
Cytosine Senescence
Guanine Huntington’s disease
Huntingtin Branches of biology
Huntington Cognitive decline dementia
Mitochondria Mitochondria dysfunction
Mitochondrial
Neurodegeneration
Oxidative Stress
Pathogenesis
Pathophysiology
Polyglutamine Expansion

Semantics

Type Source Name
disease DOID chorea
disease MESH chorea
gene UNIPROT HTT
gene UNIPROT ARMC9
gene UNIPROT AKR1A1
drug DRUGBANK Guanine
drug DRUGBANK Adenine
disease MESH oxidative stress
pathway BSID Oxidative Stress
disease MESH dementia
disease DOID dementia
disease MESH cognitive decline

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