Heterogeneous nuclear ribonucleoprotein e2 (hnrnp e2) is a component of tdp-43 aggregatesspecifically in the a and c pathological subtypes of frontotemporal lobar degeneration

Wejdan Kattuah, Boris Rogelj, Andrew King, Christopher E. Shaw, Tibor Hortobágyi, Claire Troakes

Research output: Contribution to journalArticle

1 Citation (Scopus)

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 87% 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
DOIs
Publication statusPublished - 2019

Keywords

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

ASJC Scopus subject areas

  • Neuroscience(all)

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