Article
Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease.
The Journal of cell biology - 5 Mar 2012
Hipp Mark S, Patel Chetan N, Bersuker Kirill, Riley Brigit E, Kaiser Stephen E, Shaler Thomas A, Brandeis Michael, Kopito Ron R
Abstract excerpt
Pathognomonic accumulation of ubiquitin (Ub) conjugates in human neurodegenerative diseases, such as Huntington's disease, suggests that highly aggregated proteins interfere with 26S proteasome activity. In this paper, we examine possible mechanisms by which an N-terminal fragment of mutant huntingtin (htt; N-htt) inhibits 26S function. We show that ubiquitinated N-htt-whether aggregated or not-did not choke or...
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