Dalazen Giovana Reche, Terra Melaine, Jacques Carlos Eduardo Diaz, Coelho Juliana G, Freitas Raylane, Mazzola Priscila Nicolao, Dutra-Filho Carlos Severo
Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.
Metab Brain Dis. 2014 Mar;29(1):175-83. doi: 10.1007/s11011-013-9466-3. Epub 2013 Dec 12.
Pipecolic acid (PA) levels are increased in severe metabolic disorders of the central nervous system such as Zellweger syndrome, infantile Refsum disease, neonatal adrenoleukodystrophy and hyperlysinemia. The affected individuals present progressive neurological dysfunction, hypotonia and growth retardation. The mechanisms of brain damage of these disorders remain poorly understood. Since PA catabolism can produce H2O2 by oxidases, oxidative stress may be a possible mechanism involved in the pathophysiology of these diseases. Lipoic acid (LA) is considered an efficient antioxidant and has been shown to prevent oxidative stress in experimental models of many disorders of the neurologic system. Considering that to our knowledge no study investigated the role of PA on oxidative stress, in the present work we investigated the in vitro effects of PA on some oxidative stress parameters and evaluated the LA efficacy against possible pro-oxidant effects of PA in cerebral cortex of 14-day-old rats. The activities of catalase (CAT), glutathione peroxidase (GPx), glucose 6-phosphate dehydrogenase (G6PD), and glutathione S-transferase (GST) along with reduced glutathione (GSH) content were significantly decreased, while superoxide dismutase (SOD) activity and thiobarbituric acid-reactive substances (TBA-RS) were significantly enhanced by PA. LA was able to prevent these effects by improving the activity of antioxidant enzymes, increasing GSH content and reducing TBA-RS. In contrast, glutathione reductase and 6-phosphogluconate dehydrogenase activities and sulfhydryl content were not affected. Taken together, it may be presumed that PA in vitro elicits oxidative stress and LA is able to prevent these effects.
在诸如脑肝肾综合征、婴儿型Refsum病、新生儿肾上腺脑白质营养不良和高赖氨酸血症等严重的中枢神经系统代谢紊乱中,哌可酸(PA)水平会升高。受影响的个体表现出进行性神经功能障碍、肌张力减退和生长发育迟缓。这些疾病的脑损伤机制仍知之甚少。由于PA分解代谢可通过氧化酶产生过氧化氢,氧化应激可能是这些疾病病理生理学中的一种潜在机制。硫辛酸(LA)被认为是一种有效的抗氧化剂,并且已被证明在许多神经系统疾病的实验模型中可预防氧化应激。鉴于据我们所知尚无研究调查PA对氧化应激的作用,在本研究中,我们研究了PA对一些氧化应激参数的体外影响,并评估了LA对14日龄大鼠大脑皮层中PA可能的促氧化作用的疗效。PA显著降低了过氧化氢酶(CAT)、谷胱甘肽过氧化物酶(GPx)、葡萄糖6-磷酸脱氢酶(G6PD)和谷胱甘肽S-转移酶(GST)的活性以及还原型谷胱甘肽(GSH)的含量,同时显著增强了超氧化物歧化酶(SOD)的活性和硫代巴比妥酸反应性物质(TBA-RS)。LA能够通过提高抗氧化酶的活性、增加GSH含量和降低TBA-RS来预防这些影响。相比之下,谷胱甘肽还原酶和6-磷酸葡萄糖酸脱氢酶的活性以及巯基含量未受影响。综上所述,可以推测PA在体外引发氧化应激,而LA能够预防这些影响。