Article
Long-term proteasome dysfunction in the mouse brain by expression of aberrant ubiquitin.
Neurobiology of aging - 1 Jun 2009
Fischer David F, van Dijk Renske, van Tijn Paula, Hobo Barbara, Verhage Marian C, van der Schors Roel C, Li Ka Wan, van Minnen Jan, Hol Elly M, van Leeuwen Fred W
Abstract excerpt
Many neurodegenerative diseases are characterized by deposits of ubiquitinated and aberrant proteins, suggesting a failure of the ubiquitin-proteasome system (UPS). The aberrant ubiquitin UBB(+1) is one of the ubiquitinated proteins accumulating in tauopathies such as Alzheimer's disease (AD) and polyglutamine diseases such as Huntington's disease. We have generated UBB(+1) transgenic mouse lines with post-natal...
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