Literature DB >> 22116356

Analysis of cellular localization and function of carboxy-terminal mutants of pendrin.

Aigerim Bizhanova1, Teng-Leong Chew, Satya Khuon, Peter Kopp.   

Abstract

BACKGROUND: Iodide uptake at the basolateral membrane and iodide efflux at the apical membrane of thyrocytes, essential steps in the biosynthesis of thyroid hormone, are stimulated by thyroid stimulating hormone (TSH). Pendrin (SLC26A4) is inserted into the apical membrane of thyrocytes and thought to be involved in mediating iodide efflux.
METHODS: We determined the effects of carboxy-terminal mutations of pendrin on the cellular localization and the ability to transport iodide.
RESULTS: After exposure to forskolin, the membrane abundance of wild type pendrin and iodide efflux increase. Truncation mutants lead to complete intracellular retention. Elimination of the distal part of the sulfate transporter and antisigma factor antagonist (STAS) domain with retention of the putative protein kinase A (PKA) phosphorylation site (RKDT 714-717) results in residual membrane insertion and a partial loss of function. Deletion of the PKA site results in decreased basal function and membrane insertion and abolishes the response to forskolin.
CONCLUSION: Pendrin membrane abundance and its ability to mediate iodide efflux increase after activation of the PKA pathway. Elimination of the PKA site abolishes the response to forskolin but partial basal function and membrane insertion are maintained.
Copyright © 2011 S. Karger AG, Basel.

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Year:  2011        PMID: 22116356      PMCID: PMC3709185          DOI: 10.1159/000335105

Source DB:  PubMed          Journal:  Cell Physiol Biochem        ISSN: 1015-8987


  39 in total

1.  The STAS domain - a link between anion transporters and antisigma-factor antagonists.

Authors:  L Aravind; E V Koonin
Journal:  Curr Biol       Date:  2000-01-27       Impact factor: 10.834

2.  Pendrin, the protein encoded by the Pendred syndrome gene (PDS), is an apical porter of iodide in the thyroid and is regulated by thyroglobulin in FRTL-5 cells.

Authors:  I E Royaux; K Suzuki; A Mori; R Katoh; L A Everett; L D Kohn; E D Green
Journal:  Endocrinology       Date:  2000-02       Impact factor: 4.736

3.  Polarized efflux of iodide in porcine thyrocytes occurs via a cAMP-regulated iodide channel in the apical plasma membrane.

Authors:  M Nilsson; U Björkman; R Ekholm; L E Ericson
Journal:  Acta Endocrinol (Copenh)       Date:  1992-01

4.  Prestin is the motor protein of cochlear outer hair cells.

Authors:  J Zheng; W Shen; D Z He; K B Long; L D Madison; P Dallos
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Journal:  Am J Physiol Renal Physiol       Date:  2001-02

6.  TSH regulates pendrin membrane abundance and enhances iodide efflux in thyroid cells.

Authors:  Liuska Pesce; Aigerim Bizhanova; Juan Carlos Caraballo; Whitney Westphal; Maria L Butti; Alejandro Comellas; Peter Kopp
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7.  Regulation by thyroid-stimulating hormone of sodium/iodide symporter gene expression and protein levels in FRTL-5 cells.

Authors:  T Kogai; T Endo; T Saito; A Miyazaki; A Kawaguchi; T Onaya
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8.  Pendred syndrome is caused by mutations in a putative sulphate transporter gene (PDS).

Authors:  L A Everett; B Glaser; J C Beck; J R Idol; A Buchs; M Heyman; F Adawi; E Hazani; E Nassir; A D Baxevanis; V C Sheffield; E D Green
Journal:  Nat Genet       Date:  1997-12       Impact factor: 38.330

9.  Functional differences of the PDS gene product are associated with phenotypic variation in patients with Pendred syndrome and non-syndromic hearing loss (DFNB4).

Authors:  D A Scott; R Wang; T M Kreman; M Andrews; J M McDonald; J R Bishop; R J Smith; L P Karniski; V C Sheffield
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