TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor

Mol Cell Biol. 2011 Mar;31(5):1098-108. doi: 10.1128/MCB.01279-10. Epub 2010 Dec 20.

Abstract

TDP-43, or TAR DNA-binding protein 43, is a pathological marker of a spectrum of neurodegenerative disorders, including amyotrophic lateral sclerosis and frontotemporal lobar degeneration with ubiquitin-positive inclusions. TDP-43 is an RNA/DNA-binding protein implicated in transcriptional and posttranscriptional regulation. Recent work also suggests that TDP-43 associates with cytoplasmic stress granules, which are transient structures that form in response to stress. In this study, we establish sorbitol as a novel physiological stressor that directs TDP-43 to stress granules in Hek293T cells and primary cultured glia. We quantify the association of TDP-43 with stress granules over time and show that stress granule association and size are dependent on the glycine-rich region of TDP-43, which harbors the majority of pathogenic mutations. Moreover, we establish that cells harboring wild-type and mutant TDP-43 have distinct stress responses: mutant TDP-43 forms significantly larger stress granules, and is incorporated into stress granules earlier, than wild-type TDP-43; in striking contrast, wild-type TDP-43 forms more stress granules over time, but the granule size remains relatively unchanged. We propose that mutant TDP-43 alters stress granule dynamics, which may contribute to the progression of TDP-43 proteinopathies.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / metabolism
  • Animals
  • Biological Assay
  • Cell Line
  • Cells, Cultured
  • Cytoplasmic Granules / metabolism*
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism*
  • Humans
  • Mice
  • Molecular Sequence Data
  • Mutation
  • Neuroglia / drug effects
  • Neuroglia / metabolism
  • Osmotic Pressure
  • Oxidative Stress*
  • Rats
  • Sorbitol / pharmacology*
  • TDP-43 Proteinopathies / genetics
  • TDP-43 Proteinopathies / metabolism

Substances

  • DNA-Binding Proteins
  • Sorbitol