http://purl.uniprot.org/citations/16371423 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/16371423 | http://www.w3.org/2000/01/rdf-schema#comment | "Many aggregate-prone proteins, including proteins with long polyglutamine or polyalanine tracts, cause human diseases. Polyalanine proteins may also be present in the tissue of polyglutamine diseases as a result of frameshifting of the primary polyglutamine-encoding (CAG)n repeat mutation. We have generated a Drosophila model expressing green fluorescent protein tagged to 37 alanines that manifests both toxicity and inclusion formation in various tissues. Surprisingly, we show that this aggregate-prone protein with a polyalanine expansion can also protect against polyglutamine toxicity, which can be explained by induction of heat-shock response. A heat-shock response was also seen in an oculopharyngeal muscular dystrophy mouse model expressing an authentic polyalanine-expanded protein. We also show that long polyalanines can protect against a pro-apoptotic stimulus or the toxicity caused by the long polyalanines themselves. Thus, overexpression of an aggregate-prone protein without any normal functions can result in both pathogenic and protective effects in cell culture and in vivo."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.org/dc/terms/identifier | "doi:10.1093/hmg/ddi460"xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/author | "Davies J.E."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/author | "Luo S."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/author | "Rubinsztein D.C."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/author | "Majoul I."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/author | "Pasco M.Y."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/author | "O'Kane C.J."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/author | "Berger Z."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/date | "2006"xsd:gYear |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/name | "Hum Mol Genet"xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/pages | "453-465"xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/title | "Deleterious and protective properties of an aggregate-prone protein with a polyalanine expansion."xsd:string |
http://purl.uniprot.org/citations/16371423 | http://purl.uniprot.org/core/volume | "15"xsd:string |
http://purl.uniprot.org/citations/16371423 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/16371423 |
http://purl.uniprot.org/citations/16371423 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/16371423 |
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