Heterogeneous nuclear ribonucleoprotein E2 (hnRNP E2) is a component of TDP-43 aggregates specifically in the A and C pathological subtypes of frontotemporal lobar degeneration.

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Abstract

TAR DNA-binding protein 43 (TDP-43) is the major component of the ubiquitin-positive protein aggregates seen in the majority of frontotemporal lobar degeneration and amyotrophic lateral sclerosis cases. TDP-43 belongs to the heterogeneous nuclear ribonucleoprotein (hnRNP) family that is involved in the regulation of RNA transcription, splicing, transport and translation. There are a great many hnRNPs, which often have overlapping functions and act cooperatively in RNA processing. Here we demonstrate that another hnRNP family member, hnRNP E2, shows a striking accumulation within dystrophic neurites and cytoplasmic inclusions in the frontal cortex and hippocampus of a subset of FTLD-TDP cases belonging to pathological subtypes A and C, where hnRNP E2 was found to co-localize with 85% of TDP-43 immunopositive inclusions. hnRNP E2-positive inclusions were not seen in FTLD-TDP cases with the C9orf72 expansion or in any other neurodegenerative disorders examined. This interaction with TDP-43 in specific FTLD subtypes suggests different underlying neurodegenerative pathways.
Original languageEnglish
Article number551
JournalFrontiers in Neuroscience
Volume13
Issue numberJUN
Early online date4 Jun 2019
DOIs
Publication statusE-pub ahead of print - 4 Jun 2019

Keywords

  • Als
  • Ftld
  • Hnrnp
  • Hnrnp e2
  • Tdp-43

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