The FUS about arginine methylation in ALS and FTLD

EMBO J. 2012 Nov 14;31(22):4249-51. doi: 10.1038/emboj.2012.291. Epub 2012 Oct 19.

Abstract

EMBO J (2012) 31 22, 4258–4275 doi:; DOI: 10.1038/emboj.2012.261; published online September 11 2012

In a time where links between amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) neurodegeneration are becoming increasingly clear, it is important to establish the convergent and divergent mechanisms responsible for this. Accordingly, Dormann et al (2012) have identified that methylation of the Fused in sarcoma (FUS) RGG3 domain is involved in the cytoplasmic mislocalisation of ALS-FUS mutants, through a transportin-dependent mechanism. By contrast, hypomethylation in this domain may play a role in the aberrant accumulation of FUS in FTLD-FUS. This work showcases arginine methylation as a phenomenon to watch out for in neurodegenerative pathology.

Publication types

  • Comment

MeSH terms

  • Amyotrophic Lateral Sclerosis / metabolism*
  • Arginine / metabolism*
  • Cell Nucleus / metabolism*
  • Humans
  • Karyopherins / metabolism*
  • Nuclear Localization Signals / metabolism*
  • RNA-Binding Protein FUS / metabolism*

Substances

  • Karyopherins
  • Nuclear Localization Signals
  • RNA-Binding Protein FUS
  • Arginine