Journal
FRONTIERS IN BIOSCIENCE-LANDMARK
Volume 13, Issue -, Pages 867-878Publisher
FRONTIERS IN BIOSCIENCE INC
DOI: 10.2741/2727
Keywords
TDP-43; TARDBP; alternative splicing; CFTR; Apo AII; transcription; HIV-1; SP-10; mRNA stability; NFL; frontotemporal dementia; FTD; amyotrophic lateral sclerosis; ALS; review
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Funding
- Telethon [GGP06147] Funding Source: Medline
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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.
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