| http://purl.uniprot.org/citations/28978466 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/28978466 | http://www.w3.org/2000/01/rdf-schema#comment | "Amyotrophic lateral sclerosis (ALS) is a synaptopathy accompanied by the presence of cytoplasmic aggregates containing TDP-43, an RNA-binding protein linked to ∼97% of ALS cases. Using a Drosophila model of ALS, we show that TDP-43 overexpression (OE) in motor neurons results in decreased expression of the Hsc70-4 chaperone at the neuromuscular junction (NMJ). Mechanistically, mutant TDP-43 sequesters hsc70-4 mRNA and impairs its translation. Expression of the Hsc70-4 ortholog, HSPA8, is also reduced in primary motor neurons and NMJs of mice expressing mutant TDP-43. Electrophysiology, imaging, and genetic interaction experiments reveal TDP-43-dependent defects in synaptic vesicle endocytosis. These deficits can be partially restored by OE of Hsc70-4, cysteine-string protein (Csp), or dynamin. This suggests that TDP-43 toxicity results in part from impaired activity of the synaptic CSP/Hsc70 chaperone complex impacting dynamin function. Finally, Hsc70-4/HSPA8 expression is also post-transcriptionally reduced in fly and human induced pluripotent stem cell (iPSC) C9orf72 models, suggesting a common disease pathomechanism."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.celrep.2017.09.028"xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Chou C.C."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Schwartz J.C."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Zarnescu D.C."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Levy J."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Rogers R.S."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Sattler R."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Torvund M."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Rossoll W."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Starr A."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Nishimune H."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Lorenzini I."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Coyne A.N."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Johannesmeyer J."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Zaepfel B.L."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/author | "Zinsmaier K."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/date | "2017"xsd:gYear |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/name | "Cell Rep"xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/pages | "110-125"xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/title | "Post-transcriptional Inhibition of Hsc70-4/HSPA8 Expression Leads to Synaptic Vesicle Cycling Defects in Multiple Models of ALS."xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://purl.uniprot.org/core/volume | "21"xsd:string |
| http://purl.uniprot.org/citations/28978466 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/28978466 |
| http://purl.uniprot.org/citations/28978466 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/28978466 |