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

Abstract

NOX may play an important role in the integration of the redox signal in the oxidative molecular regulation mechanism in PKU.

Prooxidant-antioxidant balance in patients with phenylketonuria and its correlation to biochemical and hematological parameters

“…However, lack of protein intake may increase the risk of nutritional defect that may result in a low total antioxidant status that can establish oxidative stress especially after adolescence [11]. Oxidative stress status is closely linked to serum Phe levels [12][13][14].…”

The oxidative molecular regulation mechanism of NOX in children with phenylketonuria

“…The interaction of NOX and other ROS‐generated system form regulatory networks and play an important role in the integration of redox signal. In our previous work, we used Pahenu2‐BTBR mice, a model mutagenized by ethylnitrosourea (ENU) with a new Alw26I restriction site in exon 7, for evaluating Phe concentrations in blood and cerebral cortex, as well as the level of ROS, mRNA and protein expression of subunits and enzyme activity of NOX (He et al, 2013; Lu et al, 2011). The results showed that all these indicators increased markedly.…”

“…In our previous work, we investigated that role of NOX in a Pahenu2‐BTBR PKU mouse model, and an in vitro cell model of PKU (Lu et al, 2011; He et al, 2013). It was found that cerebral cortical neurons subjected to an in vitro high phenylalanine insult, displayed increased superoxide production accompanied by increases of NOX protein expression and activity, which implied that NOX might be a potential therapeutic target in neuroprotective strategies against phenylalanine‐evoked oxidative brain injury in PKU.…”