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Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum
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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5427096/ https://www.ncbi.nlm.nih.gov/pubmed/27966098 http://dx.doi.org/10.1007/s12311-016-0838-1 |
Sumario: | 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. |
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