Matches in SemOpenAlex for { <https://semopenalex.org/work/W2106868839> ?p ?o ?g. }
- W2106868839 endingPage "2630" @default.
- W2106868839 startingPage "2620" @default.
- W2106868839 abstract "Mutations in the SFTPC gene associated with interstitial lung disease in human patients result in misfolding, endoplasmic reticulum (ER) retention, and degradation of the encoded surfactant protein C (SP-C) proprotein. In this study, genes specifically induced in response to transient expression of two disease-associated mutations were identified by microarray analyses. Immunoglobulin heavy chain binding protein (BiP) and two heat shock protein 40 family members, endoplasmic reticulum-localized DnaJ homologues ERdj4 and ERdj5, were significantly elevated and exhibited prolonged and specific association with the misfolded proprotein; in contrast, ERdj3 interacted with BiP, but it did not associate with either wild-type or mutant SP-C. Misfolded SP-C, ERdj4, and ERdj5 coprecipitated with p97/VCP indicating that the cochaperones remain associated with the misfolded proprotein until it is dislocated to the cytosol. Knockdown of ERdj4 and ERdj5 expression increased ER retention and inhibited degradation of misfolded SP-C, but it had little effect on the wild-type protein. Transient expression of ERdj4 and ERdj5 in X-box binding protein 1 −/− mouse embryonic fibroblasts substantially restored rapid degradation of mutant SP-C proprotein, whereas transfection of HPD mutants failed to rescue SP-C endoplasmic reticulum-associated protein degradation. ERdj4 and ERdj5 promote turnover of misfolded SP-C and this activity is dependent on their ability to stimulate BiP ATPase activity." @default.
- W2106868839 created "2016-06-24" @default.
- W2106868839 creator A5005835095 @default.
- W2106868839 creator A5029428436 @default.
- W2106868839 creator A5050169529 @default.
- W2106868839 creator A5055786810 @default.
- W2106868839 creator A5074146802 @default.
- W2106868839 date "2008-06-01" @default.
- W2106868839 modified "2023-09-27" @default.
- W2106868839 title "ERdj4 and ERdj5 Are Required for Endoplasmic Reticulum-associated Protein Degradation of Misfolded Surfactant Protein C" @default.
- W2106868839 cites W1782287000 @default.
- W2106868839 cites W1964706050 @default.
- W2106868839 cites W1966959414 @default.
- W2106868839 cites W1970090817 @default.
- W2106868839 cites W1970609612 @default.
- W2106868839 cites W1972482541 @default.
- W2106868839 cites W1981515291 @default.
- W2106868839 cites W1981551685 @default.
- W2106868839 cites W1984347348 @default.
- W2106868839 cites W1984672567 @default.
- W2106868839 cites W1988297845 @default.
- W2106868839 cites W1993446768 @default.
- W2106868839 cites W2000806214 @default.
- W2106868839 cites W2003248729 @default.
- W2106868839 cites W2006211612 @default.
- W2106868839 cites W2008559063 @default.
- W2106868839 cites W2009664225 @default.
- W2106868839 cites W2017776769 @default.
- W2106868839 cites W2024475665 @default.
- W2106868839 cites W2027716666 @default.
- W2106868839 cites W2028603831 @default.
- W2106868839 cites W2032710903 @default.
- W2106868839 cites W2034133901 @default.
- W2106868839 cites W2044235576 @default.
- W2106868839 cites W2051881845 @default.
- W2106868839 cites W2057458118 @default.
- W2106868839 cites W2064404171 @default.
- W2106868839 cites W2079465039 @default.
- W2106868839 cites W2083418232 @default.
- W2106868839 cites W2087689873 @default.
- W2106868839 cites W2094247809 @default.
- W2106868839 cites W2108412187 @default.
- W2106868839 cites W2112286580 @default.
- W2106868839 cites W2114366341 @default.
- W2106868839 cites W2117015911 @default.
- W2106868839 cites W2117257160 @default.
- W2106868839 cites W2117811962 @default.
- W2106868839 cites W2120350845 @default.
- W2106868839 cites W2122683221 @default.
- W2106868839 cites W2126953127 @default.
- W2106868839 cites W2127648442 @default.
- W2106868839 cites W2127906377 @default.
- W2106868839 cites W2129527025 @default.
- W2106868839 cites W2137755719 @default.
- W2106868839 cites W2138836503 @default.
- W2106868839 cites W2143952529 @default.
- W2106868839 cites W2145271775 @default.
- W2106868839 cites W2148134519 @default.
- W2106868839 cites W2151608616 @default.
- W2106868839 cites W2161257332 @default.
- W2106868839 cites W2169221124 @default.
- W2106868839 cites W2315096416 @default.
- W2106868839 doi "https://doi.org/10.1091/mbc.e07-07-0674" @default.
- W2106868839 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2397301" @default.
- W2106868839 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/18400946" @default.
- W2106868839 hasPublicationYear "2008" @default.
- W2106868839 type Work @default.
- W2106868839 sameAs 2106868839 @default.
- W2106868839 citedByCount "144" @default.
- W2106868839 countsByYear W21068688392012 @default.
- W2106868839 countsByYear W21068688392013 @default.
- W2106868839 countsByYear W21068688392014 @default.
- W2106868839 countsByYear W21068688392015 @default.
- W2106868839 countsByYear W21068688392016 @default.
- W2106868839 countsByYear W21068688392017 @default.
- W2106868839 countsByYear W21068688392018 @default.
- W2106868839 countsByYear W21068688392019 @default.
- W2106868839 countsByYear W21068688392020 @default.
- W2106868839 countsByYear W21068688392021 @default.
- W2106868839 countsByYear W21068688392022 @default.
- W2106868839 crossrefType "journal-article" @default.
- W2106868839 hasAuthorship W2106868839A5005835095 @default.
- W2106868839 hasAuthorship W2106868839A5029428436 @default.
- W2106868839 hasAuthorship W2106868839A5050169529 @default.
- W2106868839 hasAuthorship W2106868839A5055786810 @default.
- W2106868839 hasAuthorship W2106868839A5074146802 @default.
- W2106868839 hasBestOaLocation W21068688392 @default.
- W2106868839 hasConcept C104317684 @default.
- W2106868839 hasConcept C139447449 @default.
- W2106868839 hasConcept C143065580 @default.
- W2106868839 hasConcept C153911025 @default.
- W2106868839 hasConcept C158617107 @default.
- W2106868839 hasConcept C158619295 @default.
- W2106868839 hasConcept C204328495 @default.
- W2106868839 hasConcept C2778043179 @default.
- W2106868839 hasConcept C2779926675 @default.
- W2106868839 hasConcept C2992195973 @default.
- W2106868839 hasConcept C55493867 @default.