Article
- The EMBO Journal (2008) 27, 2725 - 2735
- doi:10.1038/emboj.2008.198
Published online: 2 October 2008
Subject Categories:
Curing of the [URE3] prion by Btn2p, a Batten disease-related protein
Dmitry S Kryndushkin1, Frank Shewmaker1 and Reed B Wickner1
- Laboratory of Biochemistry and Genetics, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA
Correspondence to:
Reed B Wickner, Laboratory of Biochemistry and Genetics, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Building 8, Room 225, 8 Center Drive, MSC0830, Bethesda, MD 20892-0830, USA. Tel.: +1 301 496 3452; Fax: +1 301 402 0240; E-mail: wickner@helix.nih.gov
Received 22 May 2008; Accepted 9 September 2008
Abstract
[URE3] is a prion (infectious protein), a self-propagating amyloid form of Ure2p, a regulator of yeast nitrogen catabolism. We find that overproduction of Btn2p, or its homologue Ypr158 (Cur1p), cures [URE3]. Btn2p is reported to be associated with late endosomes and to affect sorting of several proteins. We find that double deletion of BTN2 and CUR1 stabilizes [URE3] against curing by several agents, produces a remarkable increase in the proportion of strong [URE3] variants arising de novo and an increase in the number of [URE3] prion seeds. Thus, normal levels of Btn2p and Cur1p affect prion generation and propagation. Btn2p–green fluorescent protein (GFP) fusion proteins appear as a single dot located close to the nucleus and the vacuole. During the curing process, those cells having both Ure2p–GFP aggregates and Btn2p–RFP dots display striking colocalization. Btn2p curing requires cell division, and our results suggest that Btn2p is part of a system, reminiscent of the mammalian aggresome, that collects aggregates preventing their efficient distribution to progeny cells.
Keywords:
- amyloid,
- Hook homologue,
- Ypr158p
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