Article
Disease-specific accumulation of mutant ubiquitin as a marker for proteasomal dysfunction in the brain.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Nov 2003
Fischer David F, De Vos Rob A I, Van Dijk Renske, De Vrij Femke M S, Proper Evelien A, Sonnemans Marc A F, Verhage Marian C, Sluijs Jacqueline A, Hobo Barbara, Zouambia Mohamed, Steur Ernst N H Jansen, Kamphorst Wouter, Hol Elly M, Van Leeuwen Fred W
Abstract excerpt
Molecular misreading of the ubiquitin-B (UBB) gene results in a dinucleotide deletion in UBB mRNA. The resulting mutant protein, UBB+1, accumulates in the neuropathological hallmarks of Alzheimer disease. In vitro, UBB+1 inhibits proteasomal proteolysis, although it is also an ubiquitin fusion degradation substrate for the proteasome. Using the ligase chain reaction to detect dinucleotide deletions, we report...
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