| http://purl.uniprot.org/citations/19171884 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/19171884 | http://www.w3.org/2000/01/rdf-schema#comment | "Recent studies suggest that superoxide dismutase 1 (SOD1)-linked amyotrophic lateral sclerosis results from destabilization and misfolding of mutant forms of this abundant cytosolic enzyme. Here, we have tracked the expression and fate of a misfolding-prone human SOD1, G85R, fused to YFP, in a line of transgenic G85R SOD1-YFP mice. These mice, but not wild-type human SOD1-YFP transgenics, developed lethal paralyzing motor symptoms at 9 months. In situ RNA hybridization of spinal cords revealed predominant expression in motor neurons in spinal cord gray matter in all transgenic animals. Concordantly, G85R SOD-YFP was diffusely fluorescent in motor neurons of animals at 1 and 6 months of age, but at the time of symptoms, punctate aggregates were observed in cell bodies and processes. Biochemical analyses of spinal cord soluble extracts indicated that G85R SOD-YFP behaved as a misfolded monomer at all ages. It became progressively insoluble at 6 and 9 months of age, associated with presence of soluble oligomers observable by gel filtration. Immunoaffinity capture and mass spectrometry revealed association of G85R SOD-YFP, but not WT SOD-YFP, with the cytosolic chaperone Hsc70 at all ages. In addition, 3 Hsp110's, nucleotide exchange factors for Hsp70s, were captured at 6 and 9 months. Despite such chaperone interactions, G85R SOD-YFP formed insoluble inclusions at late times, containing predominantly intermediate filament proteins. We conclude that motor neurons, initially "compensated" to maintain the misfolded protein in a soluble state, become progressively unable to do so."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.org/dc/terms/identifier | "doi:10.1073/pnas.0813045106"xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Wang J."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Yates J.R."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Horwich A.L."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Kaufman R.J."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Feany M.B."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Farr G.W."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Furtak K."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Rutkowski D.T."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Perrin S."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Wilson J.H."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Zeiss C.J."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Ruse C.I."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/author | "Rodriguez-Gil D.J."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/date | "2009"xsd:gYear |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/name | "Proc Natl Acad Sci U S A"xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/pages | "1392-1397"xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/title | "Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS."xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://purl.uniprot.org/core/volume | "106"xsd:string |
| http://purl.uniprot.org/citations/19171884 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/19171884 |
| http://purl.uniprot.org/citations/19171884 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/19171884 |
| http://purl.uniprot.org/uniprot/P00441#attribution-A12F095274D70FBF819E583F8C40E092 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/19171884 |
| http://purl.uniprot.org/uniprot/#_A0A0A6YXF5-mappedCitation-19171884 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/19171884 |