Ferreira Gabriela K, Scaini Giselli, Jeremias Isabela C, Carvalho-Silva Milena, Gonçalves Cinara L, Pereira Talita C B, Oliveira Giovanna M T, Kist Luiza W, Bogo Maurício R, Schuck Patrícia F, Ferreira Gustavo C, Streck Emilio L
Laboratório de Bioenergética, Programa de Pós-graduação em Ciências da Saúde, Universidade do Extremo Sul Catarinense, Av. Universitária, 1105, Criciúma, 88806-000, Santa Catarina, Brazil.
Mol Neurobiol. 2014 Apr;49(2):734-40. doi: 10.1007/s12035-013-8552-1. Epub 2013 Oct 4.
Tyrosinemia type II, which is also known as Richner-Hanhart syndrome, is an inborn error of metabolism that is due to a block in the transamination reaction that converts tyrosine to p-hydroxyphenylpyruvate. Because the mechanisms of neurological dysfunction in hypertyrosinemic patients are poorly known and the symptoms of these patients are related to the central nervous system, the present study evaluated brain-derived neurotrophic factor (BDNF) levels and bdnf mRNA expression in young rats and during growth. In our acute protocol, Wistar rats (10 and 30 days old) were killed 1 h after a single intraperitoneal L-tyrosine injection (500 mg/kg) or saline. Chronic administration consisted of L-tyrosine (500 mg/kg) or saline injections 12 h apart for 24 days in Wistar rats (7 days old), and the rats were killed 12 h after the last injection. The brains were rapidly removed, and we evaluated the BDNF levels and bdnf mRNA expression. The present results showed that the acute administration of L-tyrosine decreased both BDNF and bdnf mRNA levels in the striatum of 10-day-old rats. In the 30-day-old rats, we observed decreased BDNF levels without modifications in bdnf transcript level in the hippocampus and striatum. Chronic administration of L-tyrosine increased the BDNF levels in the striatum of rats during their growth, whereas bdnf mRNA expression was not altered. We hypothesize that oxidative stress can interact with the BDNF system to modulate synaptic plasticity and cognitive function. The present results enhance our knowledge of the pathophysiology of hypertyrosinemia.
II型酪氨酸血症,也称为里什纳-汉哈特综合征,是一种先天性代谢缺陷,是由于将酪氨酸转化为对羟基苯丙酮酸的转氨反应受阻所致。由于高酪氨酸血症患者神经功能障碍的机制尚不清楚,且这些患者的症状与中枢神经系统有关,因此本研究评估了幼鼠及其生长过程中脑源性神经营养因子(BDNF)水平和bdnf mRNA表达。在我们的急性实验方案中,给Wistar大鼠(10日龄和30日龄)腹腔注射一次L-酪氨酸(500 mg/kg)或生理盐水1小时后处死。慢性给药是在7日龄的Wistar大鼠中每隔12小时注射L-酪氨酸(500 mg/kg)或生理盐水,共注射24天,最后一次注射12小时后处死大鼠。迅速取出大脑,评估BDNF水平和bdnf mRNA表达。目前的结果表明,急性给予L-酪氨酸可降低10日龄大鼠纹状体中的BDNF和bdnf mRNA水平。在30日龄大鼠中,我们观察到海马体和纹状体中BDNF水平降低,而bdnf转录水平未发生改变。慢性给予L-酪氨酸可增加大鼠生长过程中纹状体中的BDNF水平,而bdnf mRNA表达未改变。我们推测氧化应激可能与BDNF系统相互作用,以调节突触可塑性和认知功能。目前的结果增强了我们对高酪氨酸血症病理生理学的认识。