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2. Introduction
2.1. The TARDBP gene and its expression
2.2. The TDP-43 protein
2.3. TDP-43 cellular distribution
3. Functional roles of TDP-43 in transcriptional and splicing regulation
3.1. Transcriptional regulation
3.1.1. HIV-1 TAR DNA regulatory region
3.1.2. The mouse SP-10 gene promoter
3.2. Splicing regulation
3.2.1. CFTR exon 9
3.2.2. Apo AII Exon 3
3.3. mRNA stability
4. Involvement of TDP-43 in neurodegenerative disorders
5. Miscellaneous processes
6. Conclusions and perspectives
7.Acknowledgements
8.References

1. ABSTRACT

TDP-43 is a RNA/DNA binding protein that structurally resembles a typical hnRNP protein family member and displays a significant specificity for binding the common microsatellite region (GU/GT)n. Initially described as a regulator of HIV-1 gene expression, it has been reported in the past to affect both normal and pathological RNA splicing events. In particular, it has been shown to play a fundamental role in the occurrence of several monosymptomatic/full forms of Cystic Fibrosis caused by pathological skipping of CFTR exon 9 from the mature mRNA. Recently, and in a way probably unrelated to splicing, a hyperphosphorylated form of TDP-43 has also been found to accumulate in the cytoplasm of neuronal cells of patients affected by fronto temporal lobar degenerations. In addition to its role in transcription and splicing regulation, a growing body of evidence indirectly suggests that TDP-43 may be involved in other cellular processes such as microRNA biogenesis, apoptosis, and cell division. The aim of this work is to provide the basic facts about TDP-43 an assessment of the multiple functions ascribed to this protein.