| http://purl.uniprot.org/citations/26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26336106 | http://www.w3.org/2000/01/rdf-schema#comment | "Correcting the processing of ΔF508-CFTR, the most common mutation in cystic fibrosis, is the major goal in the development of new therapies for this disease. Here, we determined whether ΔF508 could be rescued by a combination of small-molecule correctors, and identified the mechanism by which correctors rescue the trafficking mutant of cystic fibrosis transmembrane conductance regulator (CFTR). We transfected COS-7 cells with ΔF508, created HEK-293 stably expressing ΔF508, and utilized CFBE41o(-) cell lines stably transduced with ΔF508. As shown previously, ΔF508 expressed less protein, was unstable at physiological temperature, and rapidly degraded. When the cells were treated with the combination C18 + C4 the mature C-band was expressed at the cell surface. After treatment with C18 + C4, we saw a lower rate of protein disappearance after translation was stopped with cycloheximide. To understand how this rescue occurs, we evaluated the change in the binding of proteins involved in endoplasmic reticulum-associated degradation, such as Hsp27 (HspB1) and Hsp40 (DnaJ). We saw a dramatic reduction in binding to heat shock proteins 27 and 40 following combined corrector therapy. siRNA experiments confirmed that a reduction in Hsp27 or Hsp40 rescued CFTR in the ΔF508 mutant, but the rescue was not additive or synergistic with C4 + 18 treatment, indicating these correctors shared a common pathway for rescue involving a network of endoplasmic reticulum-associated degradation proteins."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.m115.671925"xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/author | "Guggino W.B."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/author | "Cebotaru L."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/author | "Morales M.M."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/author | "Boinot C."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/author | "Sabirzhanova I."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/author | "Lopes-Pacheco M."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/date | "2015"xsd:gYear |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/name | "J Biol Chem"xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/pages | "25636-25645"xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/title | "Combination of Correctors Rescue DeltaF508-CFTR by Reducing Its Association with Hsp40 and Hsp27."xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://purl.uniprot.org/core/volume | "290"xsd:string |
| http://purl.uniprot.org/citations/26336106 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/26336106 |
| http://purl.uniprot.org/citations/26336106 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A089NFM8-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A089PPW6-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A088T1A1-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A096ZUB9-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A096ZUC0-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A096ZUC4-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A096ZUH4-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A096ZUB3-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |
| http://purl.uniprot.org/uniprot/#_A0A096ZUB7-mappedCitation-26336106 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26336106 |