Mannion Niamh, Arieti Fabiana, Gallo Angela, Keegan Liam P, O'Connell Mary A
Paul O'Gorman Leukaemia Research Centre, Institute of Cancer Sciences, College of Medical, Veterinary and Life Sciences, University of Glasgow, 21 Shelley Road, Glasgow G12 0ZD, UK.
CEITEC-Central European Institute of Technology, Masaryk University, Kamenice 5, Brno 625 00, Czech Republic.
Biomolecules. 2015 Sep 30;5(4):2338-62. doi: 10.3390/biom5042338.
The ADAR proteins deaminate adenosine to inosine in double-stranded RNA which is one of the most abundant modifications present in mammalian RNA. Inosine can have a profound effect on the RNAs that are edited, not only changing the base-pairing properties, but can also result in recoding, as inosine behaves as if it were guanosine. In mammals there are three ADAR proteins and two ADAR-related proteins (ADAD) expressed. All have a very similar modular structure; however, both their expression and biological function differ significantly. Only two of the ADAR proteins have enzymatic activity. However, both ADAR and ADAD proteins possess the ability to bind double-strand RNA. Mutations in ADARs have been associated with many diseases ranging from cancer, innate immunity to neurological disorders. Here, we will discuss in detail the domain structure of mammalian ADARs, the effects of RNA editing, and the role of ADARs in human diseases.
ADAR蛋白可将双链RNA中的腺苷脱氨生成肌苷，这是哺乳动物RNA中最丰富的修饰之一。肌苷对被编辑的RNA有深远影响，不仅会改变碱基配对特性，还可能导致重新编码，因为肌苷的行为类似于鸟苷。在哺乳动物中，有三种ADAR蛋白和两种ADAR相关蛋白（ADAD）表达。它们都具有非常相似的模块化结构；然而，它们的表达和生物学功能却有显著差异。只有两种ADAR蛋白具有酶活性。不过，ADAR和ADAD蛋白都具有结合双链RNA的能力。ADARs中的突变与许多疾病有关，从癌症、先天免疫到神经紊乱。在这里，我们将详细讨论哺乳动物ADARs的结构域结构、RNA编辑的影响以及ADARs在人类疾病中的作用。
