FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2155504705> ?p ?o ?g. }

• W2155504705 endingPage "609" @default.
• W2155504705 startingPage "597" @default.
• W2155504705 abstract "Multiple mutations in cystic fibrosis transmembrane conductance regulator (CFTR) impair its exit from the endoplasmic reticulum (ER). We compared two processing mutants: ΔF508 and the ER exit code mutant DAA. Although both have severe kinetic processing defect, DAA but not ΔF508 has substantial accumulation in its mature form, leading to higher level of processing at the steady state. DAA has much less profound conformational abnormalities. It has lower Hsp70 association and higher post-ER stability than ΔF508. The ER exit code is necessary for ΔF508 residual export and rescue. R555K, a mutation that rescues ΔF508 misprocessing, improves Sec24 association and enhances its post-ER stability. Using in situ limited proteolysis, we demonstrated a clear change in trypsin sensitivity in ΔF508 NBD1, which is reversed, together with that of other domains, by low temperature, R555K or both. We observed a conversion of the proteolytic pattern of DAA from the one resembling ΔF508 to the one similar to wild-type CFTR during its maturation. Low temperature and R555K are additive in improving ΔF508 conformational maturation and processing. Our data reveal a dual contribution of ER exit code and domain conformation to CFTR misprocessing and underscore the importance of conformational repair in effective rescue of ΔF508." @default.
• W2155504705 created "2016-06-24" @default.
• W2155504705 creator A5025930138 @default.
• W2155504705 creator A5033505915 @default.
• W2155504705 creator A5040205022 @default.
• W2155504705 creator A5058227867 @default.
• W2155504705 date "2010-02-15" @default.
• W2155504705 modified "2023-09-26" @default.
• W2155504705 title "Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue" @default.
• W2155504705 cites W120703973 @default.
• W2155504705 cites W1602887917 @default.
• W2155504705 cites W1677528798 @default.
• W2155504705 cites W1955928122 @default.
• W2155504705 cites W1975971473 @default.
• W2155504705 cites W1976507289 @default.
• W2155504705 cites W1980731449 @default.
• W2155504705 cites W1996779865 @default.
• W2155504705 cites W1999538527 @default.
• W2155504705 cites W1999746307 @default.
• W2155504705 cites W2001530819 @default.
• W2155504705 cites W2008149441 @default.
• W2155504705 cites W2008844827 @default.
• W2155504705 cites W2012040490 @default.
• W2155504705 cites W2015199039 @default.
• W2155504705 cites W2015357935 @default.
• W2155504705 cites W2016265972 @default.
• W2155504705 cites W2016446741 @default.
• W2155504705 cites W2018414056 @default.
• W2155504705 cites W2020741020 @default.
• W2155504705 cites W2029152767 @default.
• W2155504705 cites W2029983725 @default.
• W2155504705 cites W2039599894 @default.
• W2155504705 cites W2040690917 @default.
• W2155504705 cites W2049696186 @default.
• W2155504705 cites W2050662826 @default.
• W2155504705 cites W2059075829 @default.
• W2155504705 cites W2059953271 @default.
• W2155504705 cites W2064081526 @default.
• W2155504705 cites W2065444910 @default.
• W2155504705 cites W2067308664 @default.
• W2155504705 cites W2070043042 @default.
• W2155504705 cites W2075257287 @default.
• W2155504705 cites W2079234445 @default.
• W2155504705 cites W2082102563 @default.
• W2155504705 cites W2084491822 @default.
• W2155504705 cites W2088118742 @default.
• W2155504705 cites W2092356580 @default.
• W2155504705 cites W2093904931 @default.
• W2155504705 cites W2096639179 @default.
• W2155504705 cites W2097052321 @default.
• W2155504705 cites W2099245798 @default.
• W2155504705 cites W2117819061 @default.
• W2155504705 cites W2118445531 @default.
• W2155504705 cites W2125460059 @default.
• W2155504705 cites W2127402429 @default.
• W2155504705 cites W2130619361 @default.
• W2155504705 cites W2136324909 @default.
• W2155504705 cites W2137704498 @default.
• W2155504705 cites W2141857608 @default.
• W2155504705 cites W2146335990 @default.
• W2155504705 cites W2150796852 @default.
• W2155504705 cites W2158410448 @default.
• W2155504705 cites W2159997339 @default.
• W2155504705 cites W2160160194 @default.
• W2155504705 cites W2167190425 @default.
• W2155504705 cites W2170370146 @default.
• W2155504705 cites W2480651711 @default.
• W2155504705 doi "https://doi.org/10.1091/mbc.e09-05-0427" @default.
• W2155504705 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2820424" @default.
• W2155504705 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/20032308" @default.
• W2155504705 hasPublicationYear "2010" @default.
• W2155504705 type Work @default.
• W2155504705 sameAs 2155504705 @default.
• W2155504705 citedByCount "31" @default.
• W2155504705 countsByYear W21555047052012 @default.
• W2155504705 countsByYear W21555047052013 @default.
• W2155504705 countsByYear W21555047052014 @default.
• W2155504705 countsByYear W21555047052017 @default.
• W2155504705 countsByYear W21555047052019 @default.
• W2155504705 countsByYear W21555047052020 @default.
• W2155504705 countsByYear W21555047052021 @default.
• W2155504705 countsByYear W21555047052022 @default.
• W2155504705 countsByYear W21555047052023 @default.
• W2155504705 crossrefType "journal-article" @default.
• W2155504705 hasAuthorship W2155504705A5025930138 @default.
• W2155504705 hasAuthorship W2155504705A5033505915 @default.
• W2155504705 hasAuthorship W2155504705A5040205022 @default.
• W2155504705 hasAuthorship W2155504705A5058227867 @default.
• W2155504705 hasBestOaLocation W21555047052 @default.
• W2155504705 hasConcept C104317684 @default.
• W2155504705 hasConcept C12554922 @default.
• W2155504705 hasConcept C139447449 @default.
• W2155504705 hasConcept C143065580 @default.
• W2155504705 hasConcept C158617107 @default.
• W2155504705 hasConcept C158619295 @default.
• W2155504705 hasConcept C181199279 @default.
• W2155504705 hasConcept C2776938444 @default.
• W2155504705 hasConcept C2777339483 @default.

• next