Critical Role of the Proline-rich Region in Huntingtin for Aggregation and Cytotoxicity in Yeast *
Journal of Biological Chemistry, ISSN: 0021-9258, Vol: 281, Issue: 47, Page: 35608-35615
2006
- 108Citations
- 143Captures
- 1Mentions
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Example: if you select the 1-year option for an article published in 2019 and a metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019. If you select the 3-year option for the same article published in 2019 and the metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019, 2018 and 2017.
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Metrics Details
- Citations108
- Citation Indexes108
- 108
- CrossRef96
- Captures143
- Readers143
- 143
- Mentions1
- References1
- Wikipedia1
Article Description
Nine neurodegenerative diseases, such as Huntington, are caused by a polyglutamine (poly(Q)) expansion in otherwise unrelated proteins. Although poly(Q) expansion causes aggregation of the affected proteins, the protein context might determine the selective neuronal vulnerability found in each disease. Here we have report that, although expression of Huntingtin derivatives with a pathological poly(Q) expansion are innocuous in yeast, deletion of the flanking proline-rich region alters the shape and number of poly(Q) inclusions and unmasks toxic properties. Strikingly, deletion of Hsp104 increases the size of inclusions formed by expanded poly(Q) lacking the proline-rich region and abolishes toxicity. Overexpression of the chaperones Hsp104 or Hsp70 rescues growth defects in affected cells without resolving inclusions. However, aggregates formed by nontoxic Huntingtin derivatives or by toxic derivatives cured by chaperones are physically distinct from aggregates formed by toxic proteins. This study identifies the proline-rich region in Huntingtin as a profound cis -acting modulator of expanded poly(Q) toxicity and distinguishes between aggregates of toxic or non-toxic proteins.
Bibliographic Details
http://www.sciencedirect.com/science/article/pii/S002192582068108X; http://dx.doi.org/10.1074/jbc.m605558200; http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=33846025461&origin=inward; http://www.ncbi.nlm.nih.gov/pubmed/16973603; https://linkinghub.elsevier.com/retrieve/pii/S002192582068108X; http://www.jbc.org/lookup/doi/10.1074/jbc.M605558200; https://syndication.highwire.org/content/doi/10.1074/jbc.M605558200; https://dx.doi.org/10.1074/jbc.m605558200
Elsevier BV
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