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http://purl.uniprot.org/citations/16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/16371423http://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/16371423http://purl.org/dc/terms/identifier"doi:10.1093/hmg/ddi460"xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/author"Davies J.E."xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/author"Luo S."xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/author"Rubinsztein D.C."xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/author"Majoul I."xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/author"Pasco M.Y."xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/author"O'Kane C.J."xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/author"Berger Z."xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/date"2006"xsd:gYear
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/name"Hum Mol Genet"xsd:string
http://purl.uniprot.org/citations/16371423http://purl.uniprot.org/core/pages"453-465"xsd:string
http://purl.uniprot.org/citations/16371423http://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/16371423http://purl.uniprot.org/core/volume"15"xsd:string
http://purl.uniprot.org/citations/16371423http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/16371423
http://purl.uniprot.org/citations/16371423http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/16371423
http://purl.uniprot.org/uniprot/#_P22813-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423
http://purl.uniprot.org/uniprot/#_Q867H6-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423
http://purl.uniprot.org/uniprot/#_Q86BZ1-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423
http://purl.uniprot.org/uniprot/#_Q86BZ2-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423
http://purl.uniprot.org/uniprot/#_Q86BZ3-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423
http://purl.uniprot.org/uniprot/#_Q86BZ4-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423
http://purl.uniprot.org/uniprot/#_Q86BZ5-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423
http://purl.uniprot.org/uniprot/#_Q4H2F7-mappedCitation-16371423http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/16371423