Ceramides, the principal building blocks of all sphingolipids, have attracted the attention of many scientists around the world interested in developing treatments for cystic fibrosis, the most common genetic disease of Caucasians. Many years of fruitful research in this field have produced some fundamentally important, yet controversial results. Here, we aimed to summarize the current knowledge on the role of long- and very-long- chain ceramides, the most abundant species of ceramides in animal cells, in cystic fibrosis and other diseases. We also aim to explain the importance of the length of their side chain in the context of stability of transmembrane proteins through a concise synthesis of their biophysical chemistry, cell biology, and physiology. This review also addresses several remaining riddles in this field. Finally, we discuss the technical challenges associated with the analysis and quantification of ceramides. We provide the evaluation of the antibodies used for ceramide quantification and we demonstrate their lack of specificity. Results and discussion presented here will be of interest to anyone studying these enigmatic lipids.

中文翻译：

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超长链和长链神经酰胺在囊性纤维化和其他疾病中的生物化学：侧链的重要性。


神经酰胺是所有鞘脂的主要组成部分，吸引了世界各地许多对开发囊性纤维化（白种人最常见的遗传病）治疗方法感兴趣的科学家的注意力。该领域多年来卓有成效的研究产生了一些具有根本意义但又存在争议的结果。在这里，我们的目的是总结目前关于长链和超长链神经酰胺（动物细胞中最丰富的神经酰胺种类）在囊性纤维化和其他疾病中的作用的知识。我们还旨在通过对跨膜蛋白的生物物理化学、细胞生物学和生理学的简明综合，解释其侧链长度在跨膜蛋白稳定性方面的重要性。这篇评论还解决了该领域的几个遗留问题。最后，我们讨论了与神经酰胺分析和定量相关的技术挑战。我们提供了用于神经酰胺定量的抗体的评估，并证明了它们缺乏特异性。任何研究这些神秘脂质的人都会对这里提出的结果和讨论感兴趣。