Department of Neuroscience, School for Mental Health and Neuroscience, Maastricht University, PO Box 616, 6200 MD, Maastricht, The Netherlands.
Cell Mol Life Sci. 2013 Jan;70(2):181-203. doi: 10.1007/s00018-012-1038-x. Epub 2012 Jun 24.
Ceramide, the precursor of all complex sphingolipids, is a potent signaling molecule that mediates key events of cellular pathophysiology. In the nervous system, the sphingolipid metabolism has an important impact. Neurons are polarized cells and their normal functions, such as neuronal connectivity and synaptic transmission, rely on selective trafficking of molecules across plasma membrane. Sphingolipids are abundant on neural cellular membranes and represent potent regulators of brain homeostasis. Ceramide intracellular levels are fine-tuned and alteration of the sphingolipid-ceramide profile contributes to the development of age-related, neurological and neuroinflammatory diseases. The purpose of this review is to guide the reader towards a better understanding of the sphingolipid-ceramide pathway system. First, ceramide biology is presented including structure, physical properties and metabolism. Second, we describe the function of ceramide as a lipid second messenger in cell physiology. Finally, we highlight the relevance of sphingolipids and ceramide in the progression of different neurodegenerative diseases.
神经酰胺是所有复杂神经鞘脂的前体，是一种有效的信号分子，介导细胞病理生理学的关键事件。在神经系统中，神经鞘脂代谢具有重要影响。神经元是极化细胞，其正常功能，如神经元连接和突触传递，依赖于分子穿过质膜的选择性运输。神经酰胺在神经细胞膜上含量丰富，是大脑内稳态的有力调节者。神经酰胺细胞内水平受到精细调节，神经鞘脂-神经酰胺谱的改变有助于与年龄相关的、神经和神经炎症性疾病的发展。本文综述的目的是引导读者更好地了解神经鞘脂-神经酰胺途径系统。首先，介绍了神经酰胺的生物学特性，包括结构、物理性质和代谢。其次，描述了神经酰胺作为细胞生理学中脂质第二信使的功能。最后，强调了神经鞘脂和神经酰胺在不同神经退行性疾病进展中的相关性。
