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 language | English |
---|---|
Pages (from-to) | 746-750 |
Number of pages | 5 |
Journal | Cerebellum |
Volume | 16 |
Issue number | 3 |
DOIs | |
Publication status | Published - 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