Comparison of oxidative stress markers in classical Rett versus Zappella Rett variant.

<p>In classical Rett (RTT) patients (N = 2), all the examined oxidative stress (OS) markers were significantly increased compared to healthy controls (N = 15, all females, mean age 36.5±4.2), whereas Zappella Rett variant (Z-RTT) patients (N = 2) behave as controls subjects except for plasma 4-HNE-PAs. Intra-erythrocyte and plasma non-protein bound iron (NPBI) are markers of hypoxia with hemoglobin oxidation and subsequent heme iron release. Plasma 4-HNE PAs is a marker of protein oxidation due to aldehyde binding from lipid peroxidation sources. F(2)-isoprostanes (F₂-IsoPs) are the end-products of arachidonic acid oxidation, a polyunsaturated fatty acid that is abundant in both brain grey and white matter. F(2)-dihomo-isoprostanes (F₂-dihomo-IsoPs) derive from oxidation of adrenic acid, a fatty acid abundant in white matter, specifically myelin. F(4)-neuroprostanes (F₄-NeuroPs) are the end-products of docosahexanoic acid, abundant in neuronal membranes. Statistical differences were evaluated using Mann-Whitney sum rank test, Kruskal-Wallis analysis of variance (ANOVA) Two-tailed P-values are shown. Values are expressed as means ± standard error means (SEM); intra-erythrocyte NPBI is reported as nmol/ml erythrocytes suspension; plasma 4-HNE-PAs are expressed as arbitrary units (AU), while isoprostanes (IsoPs) are expressed as pg/ml.