Evidence of oxidative stress in very long chain fatty acid – Treated oligodendrocytes and potentialization of ROS production using RNA interference-directed knockdown of ABCD1 and ACOX1 peroxisomal proteins

Detection and Quantification of Free Radicals in Peroxisomal Disorders: A Comparative Study with Oxidative Stress Parameters

“…These results coincide with other studies that reported decreased levels of SOD in patients with peroxisomal disorders [4,19,26]. On the other hand, Petrillo et al reported similar SOD activity in peroxisomal disorder patients and control group [23], while Baarine et al, reported higher SOD in patients with peroxisomal disorders [10]. Over expression of SOD was also reported in oxidative stress conditions associated with neural disorders [30].…”

“…Elevation of VLCFA levels in the form of C26:0 levels, C24/C22 and C26/ C22 ratios are consistent with peroxisomal fatty acid metabolism disorders [9]. It is speculated that accumulation of VLCFA in peroxisomal disorders patients may be associated with oxidative stress and overproduction of free radicals, so therapeutic plans that are directed to decrease accumulation of VLCFA, reduce ROS production and increase anti-oxidant enzymes concentration and activity will significantly improve disease symptoms and quality of life in these patients [10].…”

Structure-Function Analysis of Peroxisomal ATP-binding Cassette Transporters Using Chimeric Dimers

“…Fatty acid content were analyzed as pentafluorobenzyl esters by gas chromatography/mass spectrometry in negative chemical ionization mode as described previously (30).…”

Molecular Mechanisms and Physiological Significance of Organelle Interactions and Cooperation

“…Unfortunately, these experimental details are rarely documented in detail. Above 20 µM, C24:0, and C26:0 appear to be toxic, and a loss of mitochondrial potential was seen in 158N murine oligodendrocytes, rat C6 glioma cells, rat primary neuronal-glial cells, and rat primary oligodendrocytes (Baarine et al, 2012a). This toxicity was accompanied by an increased production of mitochondrial O…”