Matches in SemOpenAlex for { <https://semopenalex.org/work/W1964736632> ?p ?o ?g. }
- W1964736632 endingPage "450" @default.
- W1964736632 startingPage "435" @default.
- W1964736632 abstract "SLC26A4/PDS mutations cause Pendred Syndrome and non-syndromic deafness. but some aspects of function and regulation of the SLC26A4 polypeptide gene product, pendrin, remain controversial or incompletely understood. We have therefore extended the functional analysis of wildtype and mutant pendrin in Xenopus oocytes, with studies of isotopic flux, electrophysiology, and protein localization. Pendrin mediated electroneutral, pH-insensitive, DIDS-insensitive anion exchange, with extracellular K((1/2)) (in mM) of 1.9 (Cl(-)), 1.8 (I(-)), and 0.9 (Br(-)). The unusual phenotype of Pendred Syndrome mutation E303Q (loss-of-function with normal surface expression) prompted systematic mutagenesis at position 303. Only mutant E303K exhibited loss-of-function unrescued by forced overexpression. Mutant E303C was insensitive to charge modification by methanethiosulfonates. The corresponding mutants SLC26A2 E336Q, SLC26A3 E293Q, and SLC26A6 E298Q exhibited similar loss-of-function phenotypes, with wildtype surface expression also documented for SLC26A2 E336Q. The strong inhibition of wildtype SLC26A2, SLC26A3, and SLC26A6 by phorbol ester contrasts with its modest inhibition of pendrin. Phorbol ester inhibition of SLC26A2, SLC26A3, and SLC26A6 was blocked by coexpressed kinase-dead PKCδ but was without effect on pendrin. Mutation of SLC26A2 serine residues conserved in PKCδ -sensitive SLC26 proteins but absent from pendrin failed to reduce PKCδ sensitivity of SLC26A2 (190)." @default.
- W1964736632 created "2016-06-24" @default.
- W1964736632 creator A5014312110 @default.
- W1964736632 creator A5037873569 @default.
- W1964736632 creator A5048846156 @default.
- W1964736632 creator A5057301762 @default.
- W1964736632 creator A5068464787 @default.
- W1964736632 creator A5076965086 @default.
- W1964736632 creator A5083322488 @default.
- W1964736632 date "2011-01-01" @default.
- W1964736632 modified "2023-10-11" @default.
- W1964736632 title "Pendrin Function and Regulation in <i>Xenopus</i> Oocytes" @default.
- W1964736632 cites W1240562150 @default.
- W1964736632 cites W1480384350 @default.
- W1964736632 cites W1555045286 @default.
- W1964736632 cites W1562080471 @default.
- W1964736632 cites W1571523472 @default.
- W1964736632 cites W1575697467 @default.
- W1964736632 cites W1591129275 @default.
- W1964736632 cites W1981699619 @default.
- W1964736632 cites W1984096889 @default.
- W1964736632 cites W1988654956 @default.
- W1964736632 cites W1988786406 @default.
- W1964736632 cites W1995499596 @default.
- W1964736632 cites W1995981655 @default.
- W1964736632 cites W2000323225 @default.
- W1964736632 cites W2001186370 @default.
- W1964736632 cites W2001258594 @default.
- W1964736632 cites W2010144039 @default.
- W1964736632 cites W2015281022 @default.
- W1964736632 cites W2023471002 @default.
- W1964736632 cites W2030323689 @default.
- W1964736632 cites W2033954000 @default.
- W1964736632 cites W2036607547 @default.
- W1964736632 cites W2037454945 @default.
- W1964736632 cites W2041216520 @default.
- W1964736632 cites W2046822015 @default.
- W1964736632 cites W2047030718 @default.
- W1964736632 cites W2047034952 @default.
- W1964736632 cites W2050987750 @default.
- W1964736632 cites W2052254874 @default.
- W1964736632 cites W2052733405 @default.
- W1964736632 cites W2071804839 @default.
- W1964736632 cites W2075951376 @default.
- W1964736632 cites W2076747420 @default.
- W1964736632 cites W2077491781 @default.
- W1964736632 cites W2082473607 @default.
- W1964736632 cites W2084338782 @default.
- W1964736632 cites W2095348084 @default.
- W1964736632 cites W2097880791 @default.
- W1964736632 cites W2101342426 @default.
- W1964736632 cites W2102180389 @default.
- W1964736632 cites W2119625302 @default.
- W1964736632 cites W2121087898 @default.
- W1964736632 cites W2122530523 @default.
- W1964736632 cites W2124235235 @default.
- W1964736632 cites W2124819816 @default.
- W1964736632 cites W2125170143 @default.
- W1964736632 cites W2126798429 @default.
- W1964736632 cites W2126996448 @default.
- W1964736632 cites W2135815268 @default.
- W1964736632 cites W2138985816 @default.
- W1964736632 cites W2146856227 @default.
- W1964736632 cites W2146941780 @default.
- W1964736632 cites W2149288036 @default.
- W1964736632 cites W2151987534 @default.
- W1964736632 cites W2156501109 @default.
- W1964736632 cites W2158410921 @default.
- W1964736632 cites W2159926333 @default.
- W1964736632 cites W2162371082 @default.
- W1964736632 cites W2170857576 @default.
- W1964736632 cites W2170873265 @default.
- W1964736632 cites W2178052495 @default.
- W1964736632 cites W2182822985 @default.
- W1964736632 cites W2183427918 @default.
- W1964736632 cites W2185791982 @default.
- W1964736632 cites W2264342144 @default.
- W1964736632 cites W4255858411 @default.
- W1964736632 doi "https://doi.org/10.1159/000335106" @default.
- W1964736632 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3709188" @default.
- W1964736632 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/22116357" @default.
- W1964736632 hasPublicationYear "2011" @default.
- W1964736632 type Work @default.
- W1964736632 sameAs 1964736632 @default.
- W1964736632 citedByCount "26" @default.
- W1964736632 countsByYear W19647366322012 @default.
- W1964736632 countsByYear W19647366322013 @default.
- W1964736632 countsByYear W19647366322014 @default.
- W1964736632 countsByYear W19647366322015 @default.
- W1964736632 countsByYear W19647366322016 @default.
- W1964736632 countsByYear W19647366322017 @default.
- W1964736632 countsByYear W19647366322018 @default.
- W1964736632 countsByYear W19647366322019 @default.
- W1964736632 countsByYear W19647366322021 @default.
- W1964736632 countsByYear W19647366322022 @default.
- W1964736632 countsByYear W19647366322023 @default.
- W1964736632 crossrefType "journal-article" @default.
- W1964736632 hasAuthorship W1964736632A5014312110 @default.