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Trace elements profile in the blood of Huntington' disease patients
Stefania Squadrone1, Paola Brizio1, Maria Cesarina Abete1
1Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, via Bologna 148, 10154 Torino, Italy.
Insights
Huntington's disease (HD) patients show altered blood metal levels. Increased iron, chromium, selenium, zinc, and arsenic may offer new diagnostic and therapeutic targets for this neurodegenerative disorder.
Area of Science:
- Neuroscience
- Biochemistry
- Genetics
Background:
- Huntington's disease (HD) is a neurodegenerative disorder characterized by motor, psychiatric, and cognitive decline.
- HD is linked to polyglutamine expansion in proteins, leading to aberrant folding.
- Metal accumulation can cause protein misfolding and oxidative stress, potentially contributing to neurodegeneration.
Purpose of the Study:
- To investigate blood concentrations of essential and nonessential trace elements in HD patients.
- To determine if metal homeostasis alterations are associated with HD pathogenesis.
Main Methods:
- Analysis of blood samples from HD patients.
- Quantification of essential trace elements (Cr, Co, Cu, Fe, Mn, Mo, Ni, Se, Zn).
- Quantification of nonessential trace elements (As, Cd, Sb, Sn, V).
Main Results:
- Elevated levels of essential elements: iron, chromium, selenium, and zinc in HD patients.
- Increased levels of the nonessential element arsenic in HD patients.
- Significant differences in blood metal profiles between HD patients and controls.
Conclusions:
- Alterations in metal homeostasis may play a role in the pathogenesis of Huntington's disease.
- Blood metal profiles could serve as a potential in vivo diagnostic and characterization tool for HD.
- Further research into metal dysregulation in HD may reveal novel therapeutic strategies.
Abstract:
Huntington' disease (HD) is an autosomal dominant neurodegenerative disease characterized by progressive motor, psychiatric, and cognitive deterioration. HD is, together with spinocerebellar ataxias, spinobulbar muscular atrophy and dentatorubral-pallido- luysian atrophy, one of the nine disorders caused by an expansion of glutamine residues in the causative protein where the polyglutamine expansion cause aberrant protein folding. Since an excessive metal's accumulation in organs may induce protein misfolding and oxidative stress, we have studied the blood concentration of essential (Cr, Co, Cu, Fe, Mn, Mo, Ni, Se, Zn) and nonessential (As, Cd, Sb, Sn, V) trace elements in HD patients. We found increased levels of the essential elements iron, chromium, selenium and zinc and of the nonessential element arsenic in the blood of HD patients. Since alteration in metals homeostasis may contribute to the pathogenesis of neurodegenerative disease and could eventually constitute a target for therapy, we may suggest the utilize of the blood metal profile as a further in vivo tool to study and characterize Huntington disease.
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