Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum

Bert M. Verheijen*, Romina J. G. Gentier, Denise J. H. P. Hermes, Fred W. van Leeuwen, David A. Hopkins

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

1 Citation (Web of Science)

Abstract

The ubiquitin-proteasome system (UPS) is one of the major mechanisms for protein breakdown in cells, targeting proteins for degradation by enzymatically conjugating them to ubiquitin molecules. Intracellular accumulation of ubiquitin-B+1 (UBB+1), a frameshift mutant of ubiquitin-B, is indicative of a dysfunctional UPS and has been implicated in several disorders, including neurodegenerative disease. UBB+1-expressing transgenic mice display widespread labeling for UBB+1 in brain and exhibit behavioral deficits. Here, we show that UBB+1 is specifically expressed in a subset of parasagittal stripes of Purkinje cells in the cerebellar cortex of a UBB+1-expressing mouse model. This expression pattern is reminiscent of that of the constitutively expressed Purkinje cell antigen HSP25, a small heat shock protein with neuroprotective properties.

Original languageEnglish
Pages (from-to)746-750
Number of pages5
JournalCerebellum
Volume16
Issue number3
DOIs
Publication statusPublished - Jun 2017

Keywords

  • Ubiquitin-proteasome system
  • Ubiquitin-B+1
  • Purkinje cell stripes
  • Zebrin II
  • Heat shock protein 25
  • Cerebellum
  • SHOCK-PROTEIN HSP25
  • ALZHEIMERS-DISEASE
  • NEURODEGENERATIVE DISEASES
  • CONSTITUTIVE EXPRESSION
  • PROTEASOMAL DEGRADATION
  • ABERRANT UBIQUITIN
  • AMYLOID PLAQUES
  • NERVOUS-SYSTEM
  • ADULT-MOUSE
  • INHIBITION

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