Matches in SemOpenAlex for { <https://semopenalex.org/work/W1973803211> ?p ?o ?g. }
- W1973803211 endingPage "33434" @default.
- W1973803211 startingPage "33423" @default.
- W1973803211 abstract "Mutations in the human kidney anion exchanger 1 (kAE1) membrane glycoprotein cause impaired urine acidification resulting in distal renal tubular acidosis (dRTA). Dominant and recessive dRTA kAE1 mutants exhibit distinct trafficking defects with retention in the endoplasmic reticulum (ER), Golgi, or mislocalization to the apical membrane in polarized epithelial cells. We examined the interaction of kAE1 with the quality control system responsible for the folding of membrane glycoproteins and the retention and degradation of misfolded mutants. Using small molecule inhibitors to disrupt chaperone interactions, two functional, dominant kAE1 mutants (R589H and R901stop), retained in the ER and targeted to the proteasome for degradation by ubiquitination, were rescued to the basolateral membrane of Madin-Darby canine kidney cells. In contrast, the Golgi-localized, recessive G701D and the severely misfolded, ER-retained dominant Southeast Asian ovalocytosis (SAO) mutants were not rescued. These results show that functional dRTA mutants are retained in the ER due to their interaction with molecular chaperones, particularly calnexin, and that disruption of these interactions can promote their escape from the ER and cell surface rescue." @default.
- W1973803211 created "2016-06-24" @default.
- W1973803211 creator A5069210461 @default.
- W1973803211 creator A5087326906 @default.
- W1973803211 date "2010-10-01" @default.
- W1973803211 modified "2023-10-12" @default.
- W1973803211 title "Cell Surface Rescue of Kidney Anion Exchanger 1 Mutants by Disruption of Chaperone Interactions" @default.
- W1973803211 cites W1484236229 @default.
- W1973803211 cites W1498086754 @default.
- W1973803211 cites W1559490361 @default.
- W1973803211 cites W1570960267 @default.
- W1973803211 cites W1580950884 @default.
- W1973803211 cites W1822034226 @default.
- W1973803211 cites W1965307365 @default.
- W1973803211 cites W1968124895 @default.
- W1973803211 cites W1971110022 @default.
- W1973803211 cites W1976507289 @default.
- W1973803211 cites W1978261013 @default.
- W1973803211 cites W1990991203 @default.
- W1973803211 cites W1995620131 @default.
- W1973803211 cites W2001143457 @default.
- W1973803211 cites W2006690805 @default.
- W1973803211 cites W2013911571 @default.
- W1973803211 cites W2038303530 @default.
- W1973803211 cites W2052588826 @default.
- W1973803211 cites W2056626899 @default.
- W1973803211 cites W2058000775 @default.
- W1973803211 cites W2064564550 @default.
- W1973803211 cites W2066284211 @default.
- W1973803211 cites W2067494371 @default.
- W1973803211 cites W2068722369 @default.
- W1973803211 cites W2073927199 @default.
- W1973803211 cites W2074492703 @default.
- W1973803211 cites W2079634353 @default.
- W1973803211 cites W2080229382 @default.
- W1973803211 cites W2080409112 @default.
- W1973803211 cites W2086213465 @default.
- W1973803211 cites W2088106795 @default.
- W1973803211 cites W2089203011 @default.
- W1973803211 cites W2090867546 @default.
- W1973803211 cites W2118160968 @default.
- W1973803211 cites W2121217914 @default.
- W1973803211 cites W2123142240 @default.
- W1973803211 cites W2126451050 @default.
- W1973803211 cites W2140407450 @default.
- W1973803211 cites W2143602939 @default.
- W1973803211 cites W2144786902 @default.
- W1973803211 cites W2145846438 @default.
- W1973803211 cites W2160021472 @default.
- W1973803211 cites W2160569176 @default.
- W1973803211 cites W2166297513 @default.
- W1973803211 cites W2167235167 @default.
- W1973803211 cites W2170550107 @default.
- W1973803211 cites W4242504587 @default.
- W1973803211 cites W4243231902 @default.
- W1973803211 cites W776803945 @default.
- W1973803211 cites W92181112 @default.
- W1973803211 doi "https://doi.org/10.1074/jbc.m110.144261" @default.
- W1973803211 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2963362" @default.
- W1973803211 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/20628050" @default.
- W1973803211 hasPublicationYear "2010" @default.
- W1973803211 type Work @default.
- W1973803211 sameAs 1973803211 @default.
- W1973803211 citedByCount "22" @default.
- W1973803211 countsByYear W19738032112012 @default.
- W1973803211 countsByYear W19738032112013 @default.
- W1973803211 countsByYear W19738032112014 @default.
- W1973803211 countsByYear W19738032112015 @default.
- W1973803211 countsByYear W19738032112016 @default.
- W1973803211 countsByYear W19738032112017 @default.
- W1973803211 countsByYear W19738032112018 @default.
- W1973803211 countsByYear W19738032112019 @default.
- W1973803211 countsByYear W19738032112020 @default.
- W1973803211 crossrefType "journal-article" @default.
- W1973803211 hasAuthorship W1973803211A5069210461 @default.
- W1973803211 hasAuthorship W1973803211A5087326906 @default.
- W1973803211 hasBestOaLocation W19738032111 @default.
- W1973803211 hasConcept C104317684 @default.
- W1973803211 hasConcept C119062480 @default.
- W1973803211 hasConcept C130361206 @default.
- W1973803211 hasConcept C134018914 @default.
- W1973803211 hasConcept C139447449 @default.
- W1973803211 hasConcept C142724271 @default.
- W1973803211 hasConcept C143065580 @default.
- W1973803211 hasConcept C144647389 @default.
- W1973803211 hasConcept C1491633281 @default.
- W1973803211 hasConcept C158617107 @default.
- W1973803211 hasConcept C158619295 @default.
- W1973803211 hasConcept C2775962898 @default.
- W1973803211 hasConcept C2776598079 @default.
- W1973803211 hasConcept C2778106830 @default.
- W1973803211 hasConcept C39587587 @default.
- W1973803211 hasConcept C41625074 @default.
- W1973803211 hasConcept C47450691 @default.
- W1973803211 hasConcept C55493867 @default.
- W1973803211 hasConcept C57581600 @default.
- W1973803211 hasConcept C71924100 @default.
- W1973803211 hasConcept C77411442 @default.