NOX, the main regulator in oxidative stress in experimental models of phenylketonuria?

作者:He, Yingzhong; Gu, Xuefan*; Lu, Lihua; Liang, Lili; Gao, Jialin; Zhang, Xinshun
来源:Journal of Pediatric Endocrinology & Metabolism, 2013, 26(7-8): 675-682.
DOI:10.1515/jpem-2012-0387

摘要

Aim: To reveal the role of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (NOX) in the integration of the redox signal in the oxidative molecular regulation mechanism in phenylketonuria (PKU). Methods: The blood samples were obtained from Pah(enu2)-BTBR PKU and wild-type mice, respectively. Phe concentration, total antioxidant capacity (T-AOC), glutathione (GSH) and maleic dialdehyde (MDA) were analyzed. After collection of the mononuclear cells, reverse transcription polymerase chain reaction (RT-PCR) for NOX was performed. In addition, NOX activity and superoxide in mononuclear cells were determined. Results: Compared to the control group, Phe concentration, T-AOC and MDA were markedly increased in PKU mice (p<0.01, p<0.05, p<0.01, respectively). However, the GSH level in PKU mice was less than that in control group (p<0.05). The mRNA level of subunits of NOX included p(47phox) and p(67phox), were increased in PKU mice (p<0.05), however, the gp91(phox) had no obvious change in the two groups (p>0.05). NOX activity and superoxide were also remarkably elevated in PKU mice (p<0.05). Conclusion : NOX may play an important role in the integration of the redox signal in the oxidative molecular regulation mechanism in PKU.