International Centre for Genetic Engineering and Biotechnology, Trieste, Italy.
RNA Biol. 2010 Jul-Aug;7(4):420-9. doi: 10.4161/rna.7.4.12205. Epub 2010 Jul 1.
Heterogeneous ribonucleoproteins (hnRNPs) are multifunctional RNA-binding proteins (RBPs) involved in many cellular processes. They participate in most gene expression pathways, from DNA replication and repair to mRNA translation. Among this class of proteins, TDP-43 (and more recently FUS/TLS) have received considerable attention due to their involvement in several neurodegenerative diseases. This finding has prompted many research groups to focus on the gene expression pathways that are regulated by these proteins. The results have uncovered a considerable complexity of TDP-43 and FUS/TLS functions due to the many independent mechanisms by which they may act to influence various cellular processes (such as DNA transcription, pre-mRNA splicing, mRNA export/import). The aim of this chapter will be to review especially some of the novel functions that have been uncovered, such as role in miRNA synthesis, regulation of transcript levels, and potential autoregulatory mechanisms in order to provide the basis for further investigations.
异质核糖核蛋白 (hnRNPs) 是多功能 RNA 结合蛋白 (RBPs)，参与许多细胞过程。它们参与了从 DNA 复制和修复到 mRNA 翻译的大多数基因表达途径。在这类蛋白质中，TDP-43（最近还有 FUS/TLS）由于参与了几种神经退行性疾病而受到了相当多的关注。这一发现促使许多研究小组专注于受这些蛋白质调节的基因表达途径。研究结果揭示了 TDP-43 和 FUS/TLS 功能的相当大的复杂性，因为它们可能通过多种独立的机制来影响各种细胞过程（如 DNA 转录、前体 mRNA 剪接、mRNA 输出/导入）。本章的目的将特别回顾一些新发现的功能，如在 miRNA 合成、转录本水平调节和潜在的自调节机制中的作用，为进一步的研究提供基础。
