2019
DOI: 10.1002/jnr.24492
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Abstract: 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 symp… Show more

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Cited by 17 publications
(8 citation statements)
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References 217 publications
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“…Mitochondrial network fragmentation provides an explanation for decreased oxidative phosphorylation observed in these mice. Misregulation of mitochondria and mitophagy are also observed in other polyglutamine expansion diseases, including HD and SBMA ( Table 1 ) ( Paul and Snyder, 2019 ; Tobore, 2019 ; Franco-Iborra et al, 2020 ; Ghosh et al, 2020 ; Naia et al, 2020 ; Pourshafie et al, 2020 ).…”
Section: Sca7 As a Mitochondrial Diseasementioning
confidence: 94%
“…Mitochondrial network fragmentation provides an explanation for decreased oxidative phosphorylation observed in these mice. Misregulation of mitochondria and mitophagy are also observed in other polyglutamine expansion diseases, including HD and SBMA ( Table 1 ) ( Paul and Snyder, 2019 ; Tobore, 2019 ; Franco-Iborra et al, 2020 ; Ghosh et al, 2020 ; Naia et al, 2020 ; Pourshafie et al, 2020 ).…”
Section: Sca7 As a Mitochondrial Diseasementioning
confidence: 94%
“…Apart from the accumulation of huntingtin, the precise mechanisms leading to neurodegeneration and subsequent clinical symptoms are not fully elucidated. Aberrations in function of glial cells, inflammation, mitochondrial dysfunction, or oxidative stress were all implicated in the pathogenesis of HD 121‐123 …”
Section: Kyna Alterations In Neurodegenerative Diseasesmentioning
confidence: 99%
“…Huntington's disease is a genetic neurodegenerative disorder characterized by the selective degeneration of neurons. This degeneration results in progressive disabilities, including motor dysfunction and both cognitive and psychiatric de iciencies [104][105][106]. HD is associated with polyglutamine-expansion; thus, the disease primarily impacts the cerebral cortex and striatum [107,108].…”
Section: Huntington's Disease (Hd)mentioning
confidence: 99%