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Article

Mutant ubiquitin found in neurodegenerative disorders is a ubiquitin fusion degradation substrate that blocks proteasomal degradation.

The Journal of cell biology - 29 Apr 2002

Lindsten Kristina, de Vrij Femke M S, Verhoef Lisette G G C, Fischer David F, van Leeuwen Fred W, Hol Elly M, Masucci Maria G, Dantuma Nico P

Abstract excerpt

Loss of neurons in neurodegenerative diseases is usually preceded by the accumulation of protein deposits that contain components of the ubiquitin/proteasome system. Affected neurons in Alzheimer's disease often accumulate UBB(+1), a mutant ubiquitin carrying a 19-amino acid C-terminal extension generated by a transcriptional dinucleotide deletion. Here we show that UBB(+1) is a potent inhibitor of...

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Identifiers

PMID: 11980917
DOI: 10.1083/jcb.200111034
PMCID: PMC2173284

Genes

UBB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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