Role of FET proteins in neurodegenerative disorders

Francesca Svetoni, Paola Frisone, Maria Paola Paronetto

Research output: Contribution to journalReview articlepeer-review

Abstract

Neurodegenerative disorders such as Alzheimer disease (AD), frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), Parkinson disease (PD), Huntington's disease (HD), and multiple sclerosis (MS) affect different neuronal cells, and have a variable age of onset, clinical symptoms, and pathological features. Despite the great progress in understanding the etiology of these disorders, the underlying mechanisms remain largely unclear. Among the processes affected in neurodegenerative diseases, alteration in RNA metabolism is emerging as a crucial player. RNA-binding proteins (RBPs) are involved at all stages of RNA metabolism and display a broad range of functions, including modulation of mRNA transcription, splicing, editing, export, stability, translation and localization and miRNA biogenesis, thus enormously impacting regulation of gene expression. On the other hand, aberrant regulation of RBP expression or activity can contribute to disease onset and progression. Recent reports identified mutations causative of neurological disorders in the genes encoding a family of RBPs named FET (FUS/TLS, EWS and TAF15). This review summarizes recent works documenting the involvement of FET proteins in the pathology of ALS, FTLD, essential tremor (ET) and other neurodegenerative diseases. Moreover, clinical implications of recent advances in FET research are critically discussed.

Original languageEnglish
Pages (from-to)1089-1102
Number of pages14
JournalRNA Biology
Volume13
Issue number11
DOIs
Publication statusPublished - Nov 1 2016

Keywords

  • ALS
  • EWS
  • FUS
  • neurodegenerative disorders
  • TAF15

ASJC Scopus subject areas

  • Molecular Biology
  • Cell Biology

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