Wilson’s disease (WD) is a rare autosomal recessive metabolic disorder resulting from mutations in the copper-transporting, P-type ATPase gene ATP7B gene, but influences of epigenetics, environment, age, and sex-related factors on the WD phenotype complicate diagnosis and clinical manifestations. Oxylipins, derivatives of omega-3, and omega-6 polyunsaturated fatty acids (PUFAs) are signaling mediators that are deeply involved in innate immunity responses; the regulation of inflammatory responses, including acute and chronic inflammation; and other disturbances related to any system diseases. Therefore, oxylipin profile tests are attractive for the diagnosis of WD. With UPLC-MS/MS lipidomics analysis, we detected 43 oxylipins in the plasma profiles of 39 patients with various clinical manifestations of WD compared with 16 healthy controls (HCs). Analyzing the similarity matrix of oxylipin profiles allowed us to cluster patients into three groups. Analysis of the data by VolcanoPlot and partial least square discriminant analysis (PLS-DA) showed that eight oxylipins and lipids stand for the variance between WD and HCs: eicosapentaenoic acid EPA, oleoylethanolamide OEA, octadecadienoic acids 9-HODE, 9-KODE, 12-hydroxyheptadecatrenoic acid 12-HHT, prostaglandins PGD2, PGE2, and 14,15-dihydroxyeicosatrienoic acids 14,15-DHET. The compounds indicate the involvement of oxidative stress damage, inflammatory processes, and peroxisome proliferator-activated receptor (PPAR) signaling pathways in this disease. The data reveal novel possible therapeutic targets and intervention strategies for treating WD.

中文翻译：

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威尔逊氏病患者血浆中的脂蛋白分布。

威尔逊氏病（WD）是一种罕见的常染色体隐性遗传代谢紊乱，是由铜转运P型ATPase基因ATP7B基因突变引起的，但是表观遗传，环境，年龄和性别相关因素对WD表型的影响使诊断和诊断变得复杂。临床表现。脂蛋白，omega-3和omega-6多不饱和脂肪酸（PUFA）的衍生物是与先天免疫反应密切相关的信号传导介质。调节炎症反应，包括急性和慢性炎症；以及与任何系统疾病有关的其他干扰。因此，脂蛋白曲线测试对于WD的诊断很有吸引力。通过UPLC-MS / MS脂质组学分析，与16例健康对照（HCs）相比，我们在39例具有各种WD临床表现的患者的血浆中检测到43种脂蛋白。通过分析脂蛋白谱的相似性矩阵，我们可以将患者分为三类。通过VolcanoPlot和偏最小二乘判别分析（PLS-DA）进行的数据分析表明，八种脂蛋白和脂质代表WD和HC之间的差异：二十碳五烯酸EPA，油酰基乙醇酰胺OEA，十八碳二烯酸9-HODE，9-KODE，12 -羟基庚二烯醛酸12-HHT，前列腺素PGD2，PGE2和14,15-二羟基二十碳三烯酸14,15-DHET。这些化合物表明该疾病涉及氧化应激损伤，炎症过程和过氧化物酶体增殖物激活受体（PPAR）信号传导途径。数据揭示了治疗WD的新的可能的治疗靶标和干预策略。