Federal University of Santa Maria, Center for Natural and Exact Sciences, Department of Biochemistry and Molecular Biology, Graduate Program in Biological Sciences: Toxicological Biochemistry, Camobi, 97105-900 Santa Maria, RS, Brazil.
Federal University of Santa Maria, Center for Natural and Exact Sciences, Department of Chemistry, Santa Maria, RS, Brazil.
Neurotoxicology. 2023 Jul;97:120-132. doi: 10.1016/j.neuro.2023.06.005. Epub 2023 Jun 10.
Copper (Cu) and Zinc (Zn) are required in small concentrations for metabolic functions, but are also toxic. There is a great concern about soil pollution by heavy metals, which may exposure the population to these toxicants, either by inhalation of dust or exposure to toxicants through ingestion of food derived from contaminated soils. In addition, the toxicity of metals in combination is questionable, as soil quality guidelines only assess them separately. It is well known that metal accumulation is often found in the pathologically affected regions of many neurodegenerative diseases, including Huntington's disease (HD). HD is caused by an autosomal dominantly inherited CAG trinucleotide repeat expansion in the huntingtin (HTT) gene. This results in the formation of a mutant huntingtin (mHTT) protein with an abnormally long polyglutamine (polyQ) repeat. The pathology of HD results in loss of neuronal cells, motor changes, and dementia. Rutin is a flavonoid found in various food sources, and previous studies indicate it has protective effects in HD models and acts as a metal chelator. However, further studies are needed to unravel its effects on metal dyshomeostasis and to discern the underlying mechanisms. In the present study, we investigated the toxic effects of long-term exposure to copper, zinc, and their mixture, and the relationship with the progression of neurotoxicity and neurodegeneration in a C. elegans-based HD model. Furthermore, we investigated the effects of rutin post metal exposure. Overall, we demonstrate that chronic exposure to the metals and their mixture altered body parameters, locomotion, and developmental delay, in addition to increasing polyQ protein aggregates in muscles and neurons causing neurodegeneration. We also propose that rutin has protective effects acting through mechanisms involving antioxidant and chelating properties. Altogether, our data provides new indications about the higher toxicity of metals in combination, the chelating potential of rutin in the C. elegans model of HD and possible strategies for future treatments of neurodegenerative diseases caused by the aggregation of proteins related to metals.
铜 (Cu) 和锌 (Zn) 是新陈代谢功能所必需的微量元素,但也具有毒性。人们非常关注重金属对土壤的污染,这可能会使人们通过吸入灰尘或摄入受污染土壤衍生的食物而接触到这些有毒物质。此外,金属混合物的毒性也存在疑问,因为土壤质量指南仅分别评估它们。众所周知,金属积累通常存在于许多神经退行性疾病的病变区域,包括亨廷顿病 (HD)。HD 是由亨廷顿 (HTT) 基因中的 CAG 三核苷酸重复序列扩增引起的常染色体显性遗传疾病。这导致形成具有异常长的多聚谷氨酰胺 (polyQ) 重复的突变型亨廷顿蛋白 (mHTT)。HD 的病理学导致神经元细胞丧失、运动变化和痴呆。芦丁是一种存在于各种食物来源中的类黄酮,先前的研究表明它对 HD 模型具有保护作用,并作为金属螯合剂。然而,需要进一步的研究来阐明其对金属动态平衡的影响,并阐明潜在的机制。在本研究中,我们研究了长期暴露于铜、锌及其混合物对秀丽隐杆线虫 HD 模型中神经毒性和神经退行性发展的毒性作用,以及暴露后芦丁的作用。总的来说,我们证明了长期暴露于金属及其混合物会改变身体参数、运动能力和发育迟缓,此外还会增加肌肉和神经元中的 polyQ 蛋白聚集体,导致神经退行性变。我们还提出,芦丁具有保护作用,其机制涉及抗氧化和螯合特性。总之,我们的数据提供了关于金属混合物毒性更高、芦丁在 HD 秀丽隐杆线虫模型中具有螯合潜力以及针对与金属相关的蛋白质聚集引起的神经退行性疾病的未来治疗策略的新指示。