Elevated Hydroperoxide Levels and Antioxidant Patterns in Papillon‐Lefèvre Syndrome

Abstract

Since the subjects with the lowest hydroperoxide contents are phenotypically healthy, whereas the affected individuals presented lower antioxidant levels and very high hydroperoxide concentrations, it has been suggested that a specific antioxidant therapy could be a promising approach in treating some PLS subjects. Moreover, unexpected manifestations of heterozygosity in the child of the third generation were also detected.

Confirmation of oxidative stress and fatty acid disturbances in two further Papillon–Lefèvre syndrome families with identification of a new mutation

“…Some disturbances in fatty acid profile were demonstrated, especially related to DHA. These findings confirm our previous findings in an unrelated Spanish family and suggest that a deficiency of CoQ 10 together with disturbance of fatty acid metabolism is a feature of PLS.…”

“…Finally, a special attention should be deserved to the possible role played by CoQ 10 since in these two new PLS families a consistent CoQ deficiency has been assessed at least in probands confirming previous findings . CoQ, on one hand, is an essential component of mitochondrial respiratory chain and, on the other hand, a key molecule for cell well‐being due to its antioxidant properties .…”

“…We previously have also reported that patients and members belonging to a same family with different grades of PLS severity showed an impaired antioxidant capacity characterized by abnormally high hydroperoxide levels and, in some cases, by altered CoQ and vitamin E contents. Moreover, an essential fatty acid deficiency (EFAD) was registered on the basis of the peculiar plasma fatty acid patterns found, with low polyunsaturated fatty acids (PUFA) and high monounsaturated fatty acids (MUFA) levels …”

Autophagy in periodontitis patients and gingival fibroblasts: unraveling the link between chronic diseases and inflammation

“…On the other hand, oxidative stress is one of the main factors explaining the pathophysiological mechanism of inflammatory conditions that occur in atherosclerosis and periodontitis. Several studies have demonstrated an increase of products from oxidative damage in plasma and serum in patients with periodontitis compared with healthy individuals [16,17]. Moreover, there is evidence both for a decreased antioxidant capacity in patients with periodontitis, evaluated by different assays [18-20].…”

Coenzyme Q10: The essential nutrient

“…Coenzyme Q10 is one of the most significant lipid antioxidants, which prevents the generation of free radicals and modifications of proteins, lipids, and DNA. In many disease conditions connected with increased generation and the action of reactive oxygen species (ROS), the concentration of coenzyme Q10 in the human body decreases[12] and the deficiency of coenzyme Q10 leads to the dysfunction of the respiratory chain, which is due to the insufficient production of highly energetic compounds, which decrease the efficiency of cells. To protect the cells and organ systems of the body against ROS, humans have evolved a highly sophisticated and complex antioxidant protection system.…”