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Skin of atopic dermatitis patients shows disturbed β-glucocerebrosidase and acid sphingomyelinase activity that relates to changes in stratum corneum lipid composition.
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids ( IF 3.9 ) Pub Date : 2020-02-21 , DOI: 10.1016/j.bbalip.2020.158673 Daphne E C Boer 1 , Jeroen van Smeden 2 , Hanin Al-Khakany 3 , Elizaveta Melnik 4 , Rianne van Dijk 3 , Samira Absalah 3 , Rob J Vreeken 5 , Caroline C P Haenen 6 , Adriana P M Lavrijsen 6 , Herman S Overkleeft 1 , Johannes M F G Aerts 1 , Joke A Bouwstra 3
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids ( IF 3.9 ) Pub Date : 2020-02-21 , DOI: 10.1016/j.bbalip.2020.158673 Daphne E C Boer 1 , Jeroen van Smeden 2 , Hanin Al-Khakany 3 , Elizaveta Melnik 4 , Rianne van Dijk 3 , Samira Absalah 3 , Rob J Vreeken 5 , Caroline C P Haenen 6 , Adriana P M Lavrijsen 6 , Herman S Overkleeft 1 , Johannes M F G Aerts 1 , Joke A Bouwstra 3
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Patients with Atopic Dermatitis (AD) suffer from inflamed skin and skin barrier defects. Proper formation of the outermost part of the skin, the stratum corneum (SC), is crucial for the skin barrier function. In this study we analyzed the localization and activity of lipid enzymes β-glucocerebrosidase (GBA) and acid sphingomyelinase (ASM) in the skin of AD patients and controls. Localization of both the expression and activity of GBA and ASM in the epidermis of AD patients was altered, particularly at lesional skin sites. These changes aligned with the altered SC lipid composition. More specifically, abnormal localization of GBA and ASM related to an increase in specific ceramide subclasses [AS] and [NS]. Moreover we related the localization of the enzymes to the amounts of SC ceramide subclasses and free fatty acids (FFAs). We report a correlation between altered localization of active GBA and ASM and a disturbed SC lipid composition. Localization of antimicrobial peptide beta-defensin-3 (HBD-3) and AD biomarker Thymus and Activation Regulated Chemokine (TARC) also appeared to be diverging in AD skin compared to control. This research highlights the relation between correct localization of expressed and active lipid enzymes and a normal SC lipid composition for a proper skin barrier.
中文翻译：
特应性皮炎患者的皮肤显示出β-葡萄糖脑苷脂酶和酸性鞘磷脂酶活性受损，这与角质层脂质组成的变化有关。
特应性皮炎（AD）患者患有皮肤发炎和皮肤屏障缺陷。皮肤最外层角质层（SC）的正确形成对于皮肤屏障功能至关重要。在这项研究中，我们分析了AD患者和对照组皮肤中脂质酶β-葡萄糖脑苷脂酶（GBA）和酸性鞘磷脂酶（ASM）的定位和活性。GBA和ASM在AD患者表皮中的表达和活性的定位都发生了变化，特别是在病变皮肤部位。这些变化与改变的SC脂质组成一致。更具体地说，GBA和ASM的异常定位与特定神经酰胺亚类[AS]和[NS]的增加有关。此外，我们将酶的定位与SC神经酰胺亚类和游离脂肪酸（FFA）的量相关。我们报告了活动的GBA和ASM的本地化更改和SC脂质成分紊乱之间的相关性。与对照相比，AD皮肤中抗菌肽β-defensin-3（HBD-3）和AD生物标志物胸腺和活化调节趋化因子（TARC）的定位也似乎有差异。这项研究强调了表达和活性脂质酶的正确定位与正常的SC脂质成分之间的关系，以形成适当的皮肤屏障。
更新日期：2020-02-21
中文翻译：
特应性皮炎患者的皮肤显示出β-葡萄糖脑苷脂酶和酸性鞘磷脂酶活性受损，这与角质层脂质组成的变化有关。
特应性皮炎（AD）患者患有皮肤发炎和皮肤屏障缺陷。皮肤最外层角质层（SC）的正确形成对于皮肤屏障功能至关重要。在这项研究中，我们分析了AD患者和对照组皮肤中脂质酶β-葡萄糖脑苷脂酶（GBA）和酸性鞘磷脂酶（ASM）的定位和活性。GBA和ASM在AD患者表皮中的表达和活性的定位都发生了变化，特别是在病变皮肤部位。这些变化与改变的SC脂质组成一致。更具体地说，GBA和ASM的异常定位与特定神经酰胺亚类[AS]和[NS]的增加有关。此外，我们将酶的定位与SC神经酰胺亚类和游离脂肪酸（FFA）的量相关。我们报告了活动的GBA和ASM的本地化更改和SC脂质成分紊乱之间的相关性。与对照相比，AD皮肤中抗菌肽β-defensin-3（HBD-3）和AD生物标志物胸腺和活化调节趋化因子（TARC）的定位也似乎有差异。这项研究强调了表达和活性脂质酶的正确定位与正常的SC脂质成分之间的关系，以形成适当的皮肤屏障。
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