Alterations in the Oxidation Products, Antioxidant Markers, Antioxidant Capacity and Lipid Patterns in Plasma of Patients Affected by Papillon-Lefèvre Syndrome

Abstract

Papillon-Lefèvre syndrome (PLS) is an uncommon disease. Less than 300 cases have been described. PLS is characterized by the association between palmar plantar hyperkeratosis (PPK) and severe precocious periodontitis that results in the premature loss of both the primary and secondary dentitions. It is known that periodontitis (PE), the destructive phase of periodontal disease, is a multifactor phenomenon involving a variety of molecular species, among them free radicals and reactive oxygen species (ROS). Antioxidants have been shown to play a critical role in modulating ROS-induced damages during PE. We wondered if patients belonging to a family group with different grades of PLS severity may present altered plasma concentrations of oxidation products as well as of lipophilic antioxidants, like Coenzyme Q or vitamin E, which are molecules that possess well-known antioxidant properties and could play a role in PE processes. We also wondered about the actual plasma total antioxidant capacity of these subjects as well as a complete identification of their plasma fatty acids features, which have been never investigated before. The results we obtained indicate an impairment in the antioxidant capacity of the subjects 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 (i.e. low PUFA, high MUFA and high delta-9 desaturase activity). This finding would support the hypothesisby Gutteridge and co-workers (Free Radic. Res. 1998, 28: 109-114) that conditions exist in which some forms of oxidative stress can lead to changes characteristic of EFAD.

Repeated edaravone treatment reduces oxidative cell damage in rat brain induced by middle cerebral artery occlusion

“…It has been reported that the concentrations of PUFA are reduced but those of monoenoic acids such as oleic acid (18:1) and palmitoleic acid (16:1) increase to compensate for the oxidative loss of PUFA under various conditions of oxidative stress. 20 Such changes in the plasma were, for example, observed in patients with adult respiratory distress syndrome, 21 multiple sclerosis, 22 Papillon-Lefevre syndrome, 23 and in newborn babies. 16 Similarly, they were noted in Long Evans Cinnamon (LEC) rats, 14 an animal model of hepatic cancer and Wilson's disease, and in rats with carbon tetrachloride poisoning.…”

Increased oxidative stress and coenzyme Q10 deficiency in juvenile fibromyalgia: amelioration of hypercholesterolemia and fatigue by ubiquinol-10 supplementation

“…A previous study reported that plasma concentrations of PUFA decreased while those of monoenoic acids, such as oleic acid (18:1) and palmitoleic acid (16:1), increased to compensate for the oxidative loss of PUFA under various conditions of oxidative stress. 24 Such changes were observed in patients with adult respiratory distress syndrome, 25 multiple sclerosis, 26 Papillon-Lefevre syndrome, 27 and in newborn babies. 28 We recently observed that an elevation of plasma FFA levels and the contents of 16:1 and 18:1 in total FFA (%16:1 and %18:1, respectively) was induced by 2-hour occlusion-reperfusion of the middle cerebral artery in rats and that this change was attenuated by the administration of a free radical scavenger drug, edaravone.…”

Metabolic Syndrome and Periodontitis: Is Oxidative Stress a Common Link?

“…Several studies have demonstrated an increase in products of oxidative damage in peri pheral blood from persons with periodontitis compared with control individuals (Battino et al, 2001;Montebugnoli et al, 2004;Baltacioglu et al, 2008). Moreover, it is evidence of a decreased anti-oxidant capacity in persons with periodontitis, evaluated by different assays (Chapple et al, 2002Battino et al, 2003;Brock et al, 2004;Panjamurthy et al, 2005;Baltacioglu et al, 2006;Akalin et al, 2007;Konopka et al, 2007;Zilinskas et al, 2007).…”