Alterations in the oxidation products, antioxidant markers, antioxidant capacity and lipid patterns in plasma of patients affected by Papillon-Lefevre syndrome
Alterations in the oxidation products, antioxidant markers, antioxidant capacity and lipid patterns in plasma of patients affected by Papillon-Lefevre syndrome
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DOI：
10.1080/1071576031000083116
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发表时间：
2003-06-01
影响因子：
3.3
通讯作者：
Bullon, P
中科院分区：
文献类型：
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作者：
Battino, M;Ferreiro, MS;Bullon, P
Papillon-Lefevre 综合征 (PLS) 是一种罕见疾病。已描述的案例不到 300 个。 PLS 的特点是掌跖角化过度 (PPK) 与严重早熟牙周炎之间的关联，导致乳牙和次生牙列过早脱落。众所周知，牙周炎（PE）是牙周病的破坏阶段，是一种涉及多种分子种类的多因素现象，其中包括自由基和活性氧（ROS）。抗氧化剂已被证明在调节 PE 期间 ROS 诱导的损伤中发挥着关键作用。我们想知道属于不同 PLS 严重程度的家庭组的患者是否可能会出现氧化产物以及亲脂性抗氧化剂（如辅酶 Q 或维生素 E ）血浆浓度的改变，这些分子具有众所周知的抗氧化特性，并且可能在 PE 过程中发挥作用。我们还想知道这些受试者的实际血浆总抗氧化能力以及对其血浆脂肪酸特征的完整鉴定，这是以前从未研究过的。我们获得的结果表明，受试者的抗氧化能力受损，其特征是氢过氧化物水平异常高，在某些情况下，辅酶 Q 和维生素 E 含量发生改变。此外，根据发现的特殊血浆脂肪酸模式（即低 PUFA、高 MUFA 和高 Delta-9 去饱和酶活性），记录了必需脂肪酸缺乏症 (EFAD)。这一发现支持 Gutteridge 及其同事的假设（Free Radic. Res. 1998, 28: 109-114），即存在某些形式的氧化应激可导致 EFAD 特征性脂肪酸模式变化的条件。
Papillon-Lefevre 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 vitantin 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 hypothesis by 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 in fatty acids patterns characteristic of EFAD.