Systemic oxidative stress in classic Rett syndrome

Assessment of oxidative stress biomarkers – neuroprostanes and dihomo-isoprostanes – in the urine of elite triathletes after two weeks of moderate-altitude training

“…Other study in cerebral tissue, observed that DHA seems to be more damaged by ischemia (restriction in blood supply to tissues) compared with hypoxia, suggesting that the increase of F 4 -NeuroPs could represent a specific marker for ischemia damage mainly [32]. In human research was indicated that acute hypoxia on Rett syndrome patients, increases plasma levels of F 4 -NeuroPs by two orders of magnitude, as compared to those of healthy controls [34]. These results supported to previous studies in vivo, about possible hypoxic state links and an increased NeuroPs values.…”

F2-dihomo-isoprostanes as potential early biomarkers of lipid oxidative damage in Rett syndrome

“…In RTT patients, we have previously shown the coexistence of hypoxia (mild chronic hypoxia) and systemic OS ( 16 ). The key biological concept, that under hypoxic conditions, OS (i.e., generation of the superoxide radical in the specifi c case) can paradoxically be generated, had been fi rst established by the work of the Rifkind group ( 47 ) by examining the in vitro hemoglobin autoxidation, which has been subsequently confi rmed ( 48,49 ).…”

“…We have previously demonstrated chronic hypoxia and/ or enhanced OS in patients with typical RTT (linked to MeCP2 gene mutations) ( 16 ), as well as in those with the early-seizure variant (ESV), linked to cyclin-dependent kinase-like 5 (CDKL5) gene mutations).…”

MtDNA mutagenesis impairs elimination of mitochondria during erythroid maturation leading to enhanced erythrocyte destruction

“…The DFO excess was removed by silica (silicagel; 25-40 mm) column chromatography. The DFO-iron complex was determined by HPLC, as previously reported 24 . In short, the flow rate was 1.5 ml min À 1 and the pressure stabilized to 115-125 bars.…”

“…Free iron is highly reactive with oxygen, and leads to a vicious circle of reactive oxygen species (ROS) generation by Fenton reaction. The free redox-active, non-protein-bound iron (NPBI) further promotes oxidative stress by oxidating, for example, polyunsaturated fatty acids of the membranes, creating reactive aldehydes, including 4-hydroxy-2-nonenal (4-HNE) 23,24 . 4-HNE forms stable adducts with amino acid residues cysteine, histidine and lysine, thereby modifying protein function 25 .…”