Article
Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum.
Cerebellum (London, England) - 1 Jun 2017
Verheijen Bert M, Gentier Romina J G, Hermes Denise J H P, van Leeuwen Fred W, Hopkins David A
Abstract excerpt
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....
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