Elsevier

The Lancet

Volume 364, Issue 9431, 24–30 July 2004, Pages 312-313
The Lancet

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Targeting energy metabolism in Huntington's disease

https://doi.org/10.1016/S0140-6736(04)16739-1Get rights and content

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    It is now known that there are strong associations between metabolic dysfunction and neurodegeneration, especially for aging disorders such as Alzheimer, Parkinson, and Huntington diseases (Procaccini et al., 2016). Therefore, metabolic manipulations have also been considered as a possible therapeutic tool for several neurodegenerative diseases, including HD (Walker and Raymond, 2004; Duan et al., 2003; Procaccini et al., 2016). Interestingly, the IKK-β pathway in the hypothalamus has also been shown to be a key for the advancement of aging in mice (Zhang et al., 2013) and aging is thought to be involved in HD pathogenesis (Machiela et al., 2020; Machiela and Southwell, 2020).

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    For HD patients, the risk for developing diabetes is nearly seven times greater than in non-HD patients (Podolsky et al., 1972). Insulin secretion is impaired, basal resting energy expenditure increases, glucose metabolism is reduced, lactate concentrations are elevated, and progressive, startling degrees of weight loss occur regardless of caloric consumption (Jenkins et al., 1993; Walker and Raymond, 2004; Weydt et al., 2006). The extreme metabolic dysfunction observed in HD patients is far from unique, however.

  • Synergistical neuroprotection of rofecoxib and statins against malonic acid induced Huntington's disease like symptoms and related cognitive dysfunction in rats

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    Huntington disease is primarily characterized by loss of medium spiny neurons in basal ganglia, which involves progressively worsening chorea followed by cognitive and psychiatric disturbances (Kent, 2004). Several studies suggest that neuro-inflammation, apoptosis, oxidative stress and mitochondrial dysfunction are widely involved in the pathophysiology of Huntington disease (Walker and Raymond, 2004; Kumar et al., 2007; Estrada Sanchez et al., 2008; Tasset et al., 2009). Malonic acid (MA) is a reversible inhibitor of succinate dehydrogenase (SDH) which induces mitochondrial dysfunction followed by secondary excitotoxicity and apoptosis due to generation of reactive oxygen species (Dedeoglu et al., 2002).

  • Etiology, Pathology, and Pathogenesis

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    It was shown that subtle weight loss is a very early hallmark of HD in humans, occurring well before onset of motor symptoms.103 There is now a consensus that weight loss in HD has a multifactorial etiology, and probably includes energy failure at the cellular level.104 Metabolite profiling of serum samples using gas chromatography/mass spectrometry found a procatabolic constellation in HD patients versus controls.105

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