Laboratório de Bioenergética, Programa de Pós-graduação em Ciências da Saúde, Universidade do Extremo Sul Catarinense, Av. Universitária, 1105, Criciúma, SC, 88806-000, Brazil.
Laboratório de Erros Inatos do Metabolismo, Programa de Pós-graduação em Ciências da Saúde, Universidade do Extremo Sul Catarinense, Criciúma, SC, Brazil.
Neurotox Res. 2018 Nov;34(4):769-780. doi: 10.1007/s12640-018-9866-6. Epub 2018 Feb 7.
Tyrosinemia type II is an inborn error of metabolism caused by a deficiency in the activity of the enzyme tyrosine aminotransferase, leading to tyrosine accumulation in the body. Although the mechanisms involved are still poorly understood, several studies have showed that higher levels of tyrosine are related to oxidative stress and therefore may affect the cholinergic system. Thus, the aim of this study was to investigate the effects of chronic administration of L-tyrosine on choline acetyltransferase activity (ChAT) and acetylcholinesterase (AChE) in the brain of rats. Moreover, we also examined the effects of one antioxidant treatment (N-acetylcysteine (NAC) + deferoxamine (DFX)) on cholinergic system. Our results showed that the chronic administration of L-tyrosine decreases the ChAT activity in the cerebral cortex, while the AChE activity was increased in the hippocampus, striatum, and cerebral cortex. Moreover, we found that the antioxidant treatment was able to prevent the decrease in the ChAT activity in the cerebral cortex. However, the increase in AChE activity induced by L-tyrosine was partially prevented the in the hippocampus and striatum, but not in the cerebral cortex. Our results also showed no differences in the aversive and spatial memory after chronic administration of L-tyrosine. In conclusion, the results of this study demonstrated an increase in AChE activity in the hippocampus, striatum, and cerebral cortex and an increase of ChAT in the cerebral cortex, without cognitive impairment. Furthermore, the alterations in the cholinergic system were partially prevented by the co-administration of NAC and DFX. Thus, the restored central cholinergic system by antioxidant treatment further supports the view that oxidative stress may be involved in the pathophysiology of tyrosinemia type II.
酪氨酸血症 II 型是一种先天性代谢缺陷病,由酪氨酸氨基转移酶活性缺乏引起,导致体内酪氨酸积聚。虽然其涉及的机制仍不清楚,但有几项研究表明,较高的酪氨酸水平与氧化应激有关,因此可能影响胆碱能系统。因此,本研究旨在探讨慢性给予 L-酪氨酸对大鼠大脑中胆碱乙酰转移酶活性(ChAT)和乙酰胆碱酯酶(AChE)的影响。此外,我们还研究了一种抗氧化剂治疗(N-乙酰半胱氨酸(NAC)+去铁胺(DFX))对胆碱能系统的影响。我们的结果表明,慢性给予 L-酪氨酸可降低大脑皮质中的 ChAT 活性,而 AChE 活性在海马、纹状体和大脑皮质中增加。此外,我们发现抗氧化剂治疗能够防止大脑皮质中 ChAT 活性的降低。然而,L-酪氨酸诱导的 AChE 活性增加在海马和纹状体中部分得到预防,但在大脑皮质中则没有。我们的研究结果还表明,慢性给予 L-酪氨酸后,厌恶和空间记忆没有差异。总之,本研究结果表明,海马、纹状体和大脑皮质中的 AChE 活性增加,大脑皮质中的 ChAT 活性增加,而认知功能没有受损。此外,NAC 和 DFX 的联合给药部分预防了胆碱能系统的改变。因此,抗氧化剂治疗恢复中枢胆碱能系统进一步支持氧化应激可能参与酪氨酸血症 II 型的病理生理学的观点。