| http://purl.uniprot.org/citations/27114530 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/27114530 | http://www.w3.org/2000/01/rdf-schema#comment | "Recent studies have indicated that mammalian cells contain a cytosolic protein disaggregation machinery comprised of Hsc70, DnaJ homologs, and Hsp110 proteins, the last of which acts to accelerate a rate-limiting step of nucleotide exchange of Hsc70. We tested the ability of transgenic overexpression of a Thy1 promoter-driven human Hsp110 protein, HspA4L (Apg1), in neuronal cells of a transgenic G85R SOD1YFP ALS mouse strain to improve survival. Notably, G85R is a mutant version of Cu/Zn superoxide dismutase 1 (SOD1) that is unable to reach native form and that is prone to aggregation, with prominent YFP-fluorescent aggregates observed in the motor neurons of the transgenic mice as early as 1 mo of age. The several-fold overexpression of Hsp110 in motor neurons of these mice was associated with an increased median survival from ∼5.5 to 7.5 mo and increased maximum survival from 6.5 to 12 mo. Improvement of survival was also observed for a G93A mutant SOD1 ALS strain. We conclude that neurodegeneration associated with cytosolic misfolding and aggregation can be ameliorated by overexpression of Hsp110, likely enhancing the function of a cytosolic disaggregation machinery."xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.org/dc/terms/identifier | "doi:10.1073/pnas.1604885113"xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/author | "Li D."xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/author | "Horwich A.L."xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/author | "Fenton W.A."xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/author | "Nagy M."xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/author | "Furtak K."xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/date | "2016"xsd:gYear |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/name | "Proc Natl Acad Sci U S A"xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/pages | "5424-5428"xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/title | "Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110."xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://purl.uniprot.org/core/volume | "113"xsd:string |
| http://purl.uniprot.org/citations/27114530 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/27114530 |
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