RNA processing pathways in amyotrophic lateral sclerosis

Neurogenetics. 2010 Jul;11(3):275-90. doi: 10.1007/s10048-010-0239-4. Epub 2010 Mar 27.


RNA processing is a tightly regulated, highly complex pathway which includes RNA transcription, pre-mRNA splicing, editing, transportation, translation, and degradation of RNA. Over the past few years, several RNA processing genes have been shown to be mutated or genetically associated with amyotrophic lateral sclerosis (ALS), including the RNA-binding proteins TDP-43 and FUS/TLS. These findings suggest that RNA processing may represent a common pathogenic mechanism involved in development of ALS. In this review, we will discuss six ALS-related, RNA processing genes including their discovery, function, and commonalities.

Publication types

  • Review

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics*
  • Animals
  • DNA Helicases
  • DNA-Binding Proteins / genetics
  • Genetic Predisposition to Disease*
  • Histone Acetyltransferases / genetics
  • Humans
  • Mice
  • Multifunctional Enzymes
  • Nerve Tissue Proteins / genetics
  • RNA Helicases / genetics
  • RNA Processing, Post-Transcriptional*
  • RNA Splicing
  • RNA-Binding Protein FUS / genetics
  • Ribonuclease, Pancreatic / genetics
  • SMN Complex Proteins / genetics
  • Transcription, Genetic


  • DNA-Binding Proteins
  • Multifunctional Enzymes
  • Nerve Tissue Proteins
  • RNA-Binding Protein FUS
  • SMN Complex Proteins
  • ELP3 protein, human
  • Histone Acetyltransferases
  • angiogenin
  • Ribonuclease, Pancreatic
  • SETX protein, human
  • DNA Helicases
  • RNA Helicases