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Gene Review

Slc26a4  -  solute carrier family 26, member 4

Mus musculus

Synonyms: Pds, Pendrin, Sodium-independent chloride/iodide transporter, Solute carrier family 26 member 4, pendrin
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Disease relevance of Slc26a4


High impact information on Slc26a4

  • We now report the cDNA cloning of CFEX, a mouse pendrin homolog with expression in the kidney by Northern analysis [6].
  • This highly discrete expression pattern is unlike that of any other known gene and involves several regions thought to be important for endolymphatic fluid resorption in the inner ear, consistent with the putative functioning of pendrin as an anion transporter [3].
  • Pds expression was detected throughout the endolymphatic duct and sac, in distinct areas of the utricle and saccule, and in the external sulcus region within the cochlea [3].
  • In situ hybridization of Foxi1(-/-) endolymphatic duct/sac epithelium shows a complete lack of the transcript encoding the chloride/iodide transporter pendrin [7].
  • These results suggest that factors that are associated with changes in distal chloride delivery govern pendrin expression in the connecting tubule and cortical collecting duct [8].

Chemical compound and disease context of Slc26a4


Biological context of Slc26a4


Anatomical context of Slc26a4

  • With moderate physiological NaCl restriction, Slc26a4 expression in the apical plasma membrane increased 2- to 3-fold in type B intercalated cells [10].
  • Endolymph volume in Slc26a4-/- mice was increased and tissue masses in areas normally occupied by type I and II fibrocytes were reduced [12].
  • Lack of pendrin expression leads to deafness and expansion of the endolymphatic compartment in inner ears of Foxi1 null mutant mice [7].
  • In type B intercalated cells, pendrin immunoreactivity was highest in apical cytoplasmic vesicles with little immunolabel along the apical plasma membrane [13].
  • BACKGROUND: Pendrin belongs to a superfamily of Cl-/anion exchangers and is expressed in the inner ear, the thyroid gland, and the kidney [14].

Associations of Slc26a4 with chemical compounds

  • However, urinary pH and Pco(2) were much lower in Slc26a4 null relative to wild-type mice due to reduced urinary buffering of secreted H(+) by HCO(3)(-) [1].
  • Excretion of ammonium, titratable acid, and citrate were the same in Slc26a4 null and wild-type mice [1].
  • Deoxycorticosterone upregulates PDS (Slc26a4) in mouse kidney: role of pendrin in mineralocorticoid-induced hypertension [5].
  • Moreover, during NaCl restriction or following DOCP treatment, Slc26a4-/- mice have a higher serum HCO3- than wild type mice from an impaired ability to excrete OH- equivalents [2].
  • Pendrin is the protein product of the PDS gene (SLC26A4) and functions in several different anion exchange modes, including chloride/formate exchange [15].

Regulatory relationships of Slc26a4


Other interactions of Slc26a4


Analytical, diagnostic and therapeutic context of Slc26a4


  1. Intercalated cell H+/OH- transporter expression is reduced in Slc26a4 null mice. Kim, Y.H., Verlander, J.W., Matthews, S.W., Kurtz, I., Shin, W., Weiner, I.D., Everett, L.A., Green, E.D., Nielsen, S., Wall, S.M. Am. J. Physiol. Renal Physiol. (2005) [Pubmed]
  2. The renal physiology of pendrin (SLC26A4) and its role in hypertension. Wall, S.M. Novartis Found. Symp. (2006) [Pubmed]
  3. Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear. Everett, L.A., Morsli, H., Wu, D.K., Green, E.D. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  4. Localization and functional studies of pendrin in the mouse inner ear provide insight about the etiology of deafness in pendred syndrome. Royaux, I.E., Belyantseva, I.A., Wu, T., Kachar, B., Everett, L.A., Marcus, D.C., Green, E.D. J. Assoc. Res. Otolaryngol. (2003) [Pubmed]
  5. Deoxycorticosterone upregulates PDS (Slc26a4) in mouse kidney: role of pendrin in mineralocorticoid-induced hypertension. Verlander, J.W., Hassell, K.A., Royaux, I.E., Glapion, D.M., Wang, M.E., Everett, L.A., Green, E.D., Wall, S.M. Hypertension (2003) [Pubmed]
  6. Identification of a chloride-formate exchanger expressed on the brush border membrane of renal proximal tubule cells. Knauf, F., Yang, C.L., Thomson, R.B., Mentone, S.A., Giebisch, G., Aronson, P.S. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  7. Lack of pendrin expression leads to deafness and expansion of the endolymphatic compartment in inner ears of Foxi1 null mutant mice. Hulander, M., Kiernan, A.E., Blomqvist, S.R., Carlsson, P., Samuelsson, E.J., Johansson, B.R., Steel, K.P., Enerbäck, S. Development (2003) [Pubmed]
  8. Pendrin regulation in mouse kidney primarily is chloride-dependent. Vallet, M., Picard, N., Loffing-Cueni, D., Fysekidis, M., Bloch-Faure, M., Deschênes, G., Breton, S., Meneton, P., Loffing, J., Aronson, P.S., Chambrey, R., Eladari, D. J. Am. Soc. Nephrol. (2006) [Pubmed]
  9. The loss of the chloride channel, ClC-5, delays apical iodide efflux and induces a euthyroid goiter in the mouse thyroid gland. van den Hove, M.F., Croizet-Berger, K., Jouret, F., Guggino, S.E., Guggino, W.B., Devuyst, O., Courtoy, P.J. Endocrinology (2006) [Pubmed]
  10. NaCl restriction upregulates renal Slc26a4 through subcellular redistribution: role in Cl- conservation. Wall, S.M., Kim, Y.H., Stanley, L., Glapion, D.M., Everett, L.A., Green, E.D., Verlander, J.W. Hypertension (2004) [Pubmed]
  11. Expression of PDS/Pds, the Pendred syndrome gene, in endometrium. Suzuki, K., Royaux, I.E., Everett, L.A., Mori-Aoki, A., Suzuki, S., Nakamura, K., Sakai, T., Katoh, R., Toda, S., Green, E.D., Kohn, L.D. J. Clin. Endocrinol. Metab. (2002) [Pubmed]
  12. Loss of KCNJ10 protein expression abolishes endocochlear potential and causes deafness in Pendred syndrome mouse model. Wangemann, P., Itza, E.M., Albrecht, B., Wu, T., Jabba, S.V., Maganti, R.J., Lee, J.H., Everett, L.A., Wall, S.M., Royaux, I.E., Green, E.D., Marcus, D.C. BMC medicine [electronic resource]. (2004) [Pubmed]
  13. Localization of pendrin in mouse kidney. Wall, S.M., Hassell, K.A., Royaux, I.E., Green, E.D., Chang, J.Y., Shipley, G.L., Verlander, J.W. Am. J. Physiol. Renal Physiol. (2003) [Pubmed]
  14. Regulation of the expression of the Cl-/anion exchanger pendrin in mouse kidney by acid-base status. Wagner, C.A., Finberg, K.E., Stehberger, P.A., Lifton, R.P., Giebisch, G.H., Aronson, P.S., Geibel, J.P. Kidney Int. (2002) [Pubmed]
  15. Formate-stimulated NaCl absorption in the proximal tubule is independent of the pendrin protein. Karniski, L.P., Wang, T., Everett, L.A., Green, E.D., Giebisch, G., Aronson, P.S. Am. J. Physiol. Renal Physiol. (2002) [Pubmed]
  16. Co-expression of pendrin, vacuolar H+-ATPase alpha4-subunit and carbonic anhydrase II in epithelial cells of the murine endolymphatic sac. Dou, H., Xu, J., Wang, Z., Smith, A.N., Soleimani, M., Karet, F.E., Greinwald, J.H., Choo, D. J. Histochem. Cytochem. (2004) [Pubmed]
  17. Localization of the ammonium transporter proteins RhBG and RhCG in mouse kidney. Verlander, J.W., Miller, R.T., Frank, A.E., Royaux, I.E., Kim, Y.H., Weiner, I.D. Am. J. Physiol. Renal Physiol. (2003) [Pubmed]
  18. Prolactin regulation of the pendrin-iodide transporter in the mammary gland. Rillema, J.A., Hill, M.A. Am. J. Physiol. Endocrinol. Metab. (2003) [Pubmed]
  19. Immunocytochemical localization of pendrin in intercalated cell subtypes in rat and mouse kidney. Kim, Y.H., Kwon, T.H., Frische, S., Kim, J., Tisher, C.C., Madsen, K.M., Nielsen, S. Am. J. Physiol. Renal Physiol. (2002) [Pubmed]
  20. Age-dependent characterization of pendrin gene expression in various tissues of deer mice. Ramachandran, B.R., Gentles, A.B., Cox, S.B., Smith, E.E. Comp. Biochem. Physiol. B, Biochem. Mol. Biol. (2006) [Pubmed]
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