Dhers L, Ducassou L, Boucher J-L, Mansuy D
UMR 8601 CNRS, Université Paris Descartes, Paris Sorbonne Cité, 45 rue des Saints Pères, 75006, Paris, France.
Cell Mol Life Sci. 2017 May;74(10):1859-1869. doi: 10.1007/s00018-016-2443-3. Epub 2017 Jan 12.
Cytochrome P450 2U1 (CYP2U1) exhibits several distinctive characteristics among the 57 human CYPs, such as its presence in almost all living organisms with a highly conserved sequence, its particular gene organization with only five exons, its major location in thymus and brain, and its protein sequence involving an unusually long N-terminal region containing 8 proline residues and an insert of about 20 amino acids containing 5 arginine residues after the transmembrane helix. Few substrates, including fatty acids, N-arachidonoylserotonin (AS), and some drugs, have been reported so far. However, its biological roles remain largely unknown, even though CYP2U1 mutations have been involved in some pathological situations, such as complicated forms of hereditary spastic paraplegia. These data together with its ability to hydroxylate some fatty acids and AS suggest its possible role in lipid metabolism.
细胞色素P450 2U1（CYP2U1）在57种人类细胞色素P450中表现出几个独特的特征，例如它几乎存在于所有生物体中，序列高度保守；其独特的基因结构仅有五个外显子；主要位于胸腺和大脑中；其蛋白质序列包含一个异常长的N端区域，有8个脯氨酸残基，并且在跨膜螺旋后有一段约20个氨基酸的插入序列，含有5个精氨酸残基。到目前为止，仅报道了少数底物，包括脂肪酸、N-花生四烯酰基血清素（AS）和一些药物。然而，尽管CYP2U1突变与一些病理情况有关，如复杂形式的遗传性痉挛性截瘫，但其生物学作用在很大程度上仍不清楚。这些数据连同其使某些脂肪酸和AS羟基化的能力表明它在脂质代谢中可能发挥作用。
