RNA-binding proteins and RNA metabolism: A new scenario in the pathogenesis of Amyotrophic Lateral Sclerosis

C. Colombrita, E. Onesto, C. Tiloca, N. Ticozzi, V. Silani, A. Ratti

Research output: Contribution to journalArticle

Abstract

Several RNA-processing genes have been implicated in the pathogenesis of Amyotrophic lateral sclerosis (ALS). In particular, causative mutations in the genes encoding for two DNA/RNA binding proteins, TAR DNA binding protein-43 (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS), were recently identified in ALS patients. These genetic findings and the presence of abnormal aggregates of these two RNA-binding proteins in ALS affected tissues suggest that molecular mechanisms regulating RNA metabolism are implicated in ALS pathogenesis through common pathways. In this review similarities and differences between TDP-43 and FUS/TLS proteins and their activities in physiological and pathological conditions will be discussed.

Original languageEnglish
Pages (from-to)83-99
Number of pages17
JournalArchives Italiennes de Biologie
Volume149
Issue number1
Publication statusPublished - 2011

Keywords

  • Fused in sarcoma/translocated in liposarcoma (FUS/TLS)
  • Protein aggregates
  • RNA-binding protein (RBP)
  • TAR DNA binding protein-43 (TDP-43)

ASJC Scopus subject areas

  • Medicine(all)
  • Cell Biology
  • Physiology

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