Laboratório de Neurologia Experimental, Programa de Pós-graduação em Ciências da Saúde, Universidade do Extremo Sul Catarinense, Av. Universitária, 1105, Criciúma, SC, 88806-000, Brazil.
Instituto Nacional de Ciência e Tecnologia Translacional em Medicina, Porto Alegre, RS, Brazil.
Metab Brain Dis. 2020 Feb;35(2):295-303. doi: 10.1007/s11011-019-00525-x. Epub 2019 Dec 11.
Tyrosinemia type II is an autosomal recessive inborn error of metabolism caused by hepatic cytosolic tyrosine aminotransferase deficiency. Importantly, this disease is associated with neurological and developmental abnormalities in many patients. Considering that the mechanisms underlying neurological dysfunction in hypertyrosinemic patients are poorly understood, in the present work we investigated the levels of cytokines - tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β), IL-6 and IL-10 - in cerebellum, hippocampus, striatum of young rats exposed to chronic administration of L-tyrosine. In addition, we also investigated the impact of the supplementation with Omega-3 fatty acids (n-3 PUFA) on the rodent model of Tyrosinemia. Notably, previous study demonstrated an association between L-tyrosine toxicity and n-3 PUFA deficiency. Our results showed a significant increase in the levels of pro- and anti-inflammatory cytokines in brain structures when animals were administered with L-tyrosine. Cerebral cortex and striatum seem to be more susceptible to the inflammation induced by tyrosine toxicity. Importantly, n-3 PUFA supplementation attenuated the alterations on cytokines levels induced by tyrosine exposure in brain regions of infant rats. In conclusion, the brain inflammation is also an important process related to tyrosine neurotoxicity observed in the experimental model of Tyrosinemia. Finally, n-3 PUFA supplementation could be considered as a potential neuroprotective adjunctive therapy for Tyrosinemias, especially type II.
酪氨酸血症 II 型是一种常染色体隐性遗传的先天性代谢缺陷病,由肝胞质酪氨酸氨基转移酶缺乏引起。重要的是,这种疾病与许多患者的神经和发育异常有关。鉴于高酪氨酸血症患者神经功能障碍的机制尚未完全了解,本研究在慢性给予 L-酪氨酸的幼年大鼠的小脑、海马和纹状体中研究了细胞因子 - 肿瘤坏死因子-α (TNF-α)、白细胞介素-1β (IL-1β)、IL-6 和 IL-10 的水平。此外,我们还研究了补充ω-3 脂肪酸 (n-3PUFA) 对酪氨酸血症啮齿动物模型的影响。值得注意的是,先前的研究表明 L-酪氨酸毒性与 n-3PUFA 缺乏之间存在关联。我们的研究结果表明,当动物给予 L-酪氨酸时,大脑结构中的促炎和抗炎细胞因子水平显著增加。大脑皮质和纹状体似乎更容易受到酪氨酸毒性引起的炎症的影响。重要的是,n-3PUFA 补充可减轻酪氨酸暴露引起的幼年大鼠脑区细胞因子水平的改变。总之,脑炎症也是观察到的酪氨酸神经毒性的实验模型中的一个重要过程。最后,n-3PUFA 补充可能被认为是酪氨酸血症的一种潜在的神经保护辅助治疗方法,特别是 II 型。