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Gene Review

SLC26A5  -  solute carrier family 26 (anion exchanger)...

Homo sapiens

Synonyms: DFNB61, PRES, Prestin, Solute carrier family 26 member 5
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Disease relevance of SLC26A5

  • Prestin, a cochlear motor protein, is defective in non-syndromic hearing loss [1].
  • PRINTO/PRES international website for families of children with rheumatic diseases: [2].
  • INTERVENTIONS: Before administering each drug, peak airway pressure (Ppeak), end inspiratory pressure (Pei), resistive pressure (Pres), and auto positive--end expiratory pressure (auto-PEEP) were measured [3].

Psychiatry related information on SLC26A5


High impact information on SLC26A5

  • Prestin is required for electromotility of the outer hair cell and for the cochlear amplifier [5].
  • Here, we show that voltage sensitivity is conferred to prestin by the intracellular anions chloride and bicarbonate [6].
  • Prestin, a transmembrane protein found in the outer hair cells of the cochlea, represents a new type of molecular motor, which is likely to be of great interest to molecular cell biologists [7].
  • Thyroid hormone is a critical determinant for the regulation of the cochlear motor protein prestin [8].
  • Prestin, a protein related to a sulfate/anion transport protein, recently has been identified and proposed as the OHC motor molecule [8].

Biological context of SLC26A5


Anatomical context of SLC26A5

  • We show both directly and indirectly that SLC26A5, rat prestin, takes up hexoses when expressed in several cell lines [12].
  • Direct measurements of labelled fructose transport into COS-7 cells expressing prestin are reported here [12].
  • The C-terminus of prestin influences nonlinear capacitance and plasma membrane targeting [13].
  • However, there is ample evidence for an alternative mode of force generation by hair cells of non-mammals, such as frogs and turtles, which probably lack prestin [14].
  • PRES applies short suction bursts that have a different impact on baroreceptors depending on their timing within the cardiac cycle and has the advantage that subjects cannot easily discriminate between conditions of stimulation and inhibition [15].

Associations of SLC26A5 with chemical compounds


Physical interactions of SLC26A5


Other interactions of SLC26A5

  • Subsequently, others and our laboratory cloned prestin, a cochlear motor protein gene (SLC26A5), a putative pancreatic anion transporter (SLC26A6), and SLC26A7-SLC26A11 [20].
  • Co-expression of KCNQ4 and prestin, the OHC motor protein, altered the voltage activation by a further -15 mV [19].
  • Since most deletion mutations eliminated plasma membrane targeting, chimeric proteins were constructed by fusing prestin, at amino acid 515 or 644, with the homologous portion of the C-terminus from the two most closely related SLC26A members, pendrin and putative anion exchanger 1 [13].
  • In outer hair cells, TH enhanced the expression of the prestin gene through TRbeta [21].
  • After analysis of these 477 clones, 50 known genes were identified, including two previously known OHC-specific proteins: oncomodulin and the recently described motor protein prestin [22].

Analytical, diagnostic and therapeutic context of SLC26A5


  1. Prestin, a cochlear motor protein, is defective in non-syndromic hearing loss. Liu, X.Z., Ouyang, X.M., Xia, X.J., Zheng, J., Pandya, A., Li, F., Du, L.L., Welch, K.O., Petit, C., Smith, R.J., Webb, B.T., Yan, D., Arnos, K.S., Corey, D., Dallos, P., Nance, W.E., Chen, Z.Y. Hum. Mol. Genet. (2003) [Pubmed]
  2. PRINTO/PRES international website for families of children with rheumatic diseases: Ruperto, N., Garcia-Munitis, P., Villa, L., Pesce, M., Aggarwal, A., Fasth, A., Avcin, T., Bae, S.C., Balogh, Z., Li, C., De Inocencio, J., Dibra, M., Dolezalova, P., Miedany, Y.E., Flato, B., Harjacek, M., Huppertz, H.I., Kanakoudi-Tsakalidou, F., Wulffraat, N., Lahdenne, P., Melo-Gomes, J.A., Mihaylova, D., Nielsen, S., Nikishina, I., Ozdogan, H., Pagava, K., Panaviene, V., Prieur, A.M., Romicka, A.M., Rumba, I., Shafaie, N., Susic, G., Takei, S., Uziel, Y., Vesely, R., Woo, P., Martini, A. Ann. Rheum. Dis. (2005) [Pubmed]
  3. Comparison of one versus two bronchodilators in ventilated COPD patients. Fernandez, A., Muñoz, J., de la Calle, B., Alia, I., Ezpeleta, A., de la Cal, M.A., Reyes, A. Intensive care medicine. (1994) [Pubmed]
  4. Effects of PRES baroreceptor stimulation on thermal and mechanical pain threshold in borderline hypertensives and normotensives. Rau, H., Brody, S., Larbig, W., Pauli, P., Vöhringer, M., Harsch, B., Kröling, P., Birbaumer, N. Psychophysiology. (1994) [Pubmed]
  5. Prestin is required for electromotility of the outer hair cell and for the cochlear amplifier. Liberman, M.C., Gao, J., He, D.Z., Wu, X., Jia, S., Zuo, J. Nature (2002) [Pubmed]
  6. Intracellular anions as the voltage sensor of prestin, the outer hair cell motor protein. Oliver, D., He, D.Z., Klöcker, N., Ludwig, J., Schulte, U., Waldegger, S., Ruppersberg, J.P., Dallos, P., Fakler, B. Science (2001) [Pubmed]
  7. Prestin, a new type of motor protein. Dallos, P., Fakler, B. Nat. Rev. Mol. Cell Biol. (2002) [Pubmed]
  8. Thyroid hormone is a critical determinant for the regulation of the cochlear motor protein prestin. Weber, T., Zimmermann, U., Winter, H., Mack, A., Köpschall, I., Rohbock, K., Zenner, H.P., Knipper, M. Proc. Natl. Acad. Sci. U.S.A. (2002) [Pubmed]
  9. Prestin, the motor protein of outer hair cells. Zheng, J., Madison, L.D., Oliver, D., Fakler, B., Dallos, P. Audiol. Neurootol. (2002) [Pubmed]
  10. Sugar transport by members of the SLC26 superfamily. Ashmore, J., Chambard, J.M. Novartis Found. Symp. (2006) [Pubmed]
  11. High frequency of the IVS2-2A>G DNA sequence variation in SLC26A5, encoding the cochlear motor protein prestin, precludes its involvement in hereditary hearing loss. Tang, H.Y., Xia, A., Oghalai, J.S., Pereira, F.A., Alford, R.L. BMC Med. Genet. (2005) [Pubmed]
  12. Sugar transport by mammalian members of the SLC26 superfamily of anion-bicarbonate exchangers. Chambard, J.M., Ashmore, J.F. J. Physiol. (Lond.) (2003) [Pubmed]
  13. The C-terminus of prestin influences nonlinear capacitance and plasma membrane targeting. Zheng, J., Du, G.G., Matsuda, K., Orem, A., Aguiñaga, S., Deák, L., Navarrete, E., Madison, L.D., Dallos, P. J. Cell. Sci. (2005) [Pubmed]
  14. Active hair bundle movements in auditory hair cells. Fettiplace, R. J. Physiol. (Lond.) (2006) [Pubmed]
  15. Baroreceptor stimulation alters cortical activity. Rau, H., Pauli, P., Brody, S., Elbert, T., Birbaumer, N. Psychophysiology. (1993) [Pubmed]
  16. N-linked glycosylation sites of the motor protein prestin: effects on membrane targeting and electrophysiological function. Matsuda, K., Zheng, J., Du, G.G., Klöcker, N., Madison, L.D., Dallos, P. J. Neurochem. (2004) [Pubmed]
  17. Voltage-induced membrane movement. Zhang, P.C., Keleshian, A.M., Sachs, F. Nature (2001) [Pubmed]
  18. Comparison of metabolic effects of orlistat and sibutramine treatment in Type 2 diabetic obese patients. Derosa, G., Cicero, A.F., Murdolo, G., Ciccarelli, L., Fogari, R. Diabetes Nutr. Metab. (2004) [Pubmed]
  19. Regulation of the voltage-gated potassium channel KCNQ4 in the auditory pathway. Chambard, J.M., Ashmore, J.F. Pflugers Arch. (2005) [Pubmed]
  20. Overview of the SLC26 family and associated diseases. Kere, J. Novartis Found. Symp. (2006) [Pubmed]
  21. Thyroid hormone receptors TRalpha1 and TRbeta differentially regulate gene expression of Kcnq4 and prestin during final differentiation of outer hair cells. Winter, H., Braig, C., Zimmermann, U., Geisler, H.S., Fränzer, J.T., Weber, T., Ley, M., Engel, J., Knirsch, M., Bauer, K., Christ, S., Walsh, E.J., McGee, J., Köpschall, I., Rohbock, K., Knipper, M. J. Cell. Sci. (2006) [Pubmed]
  22. Identification of differentially expressed cDNA clones from gerbil cochlear outer hair cells. Zheng, J., Long, K.B., Robison, D.E., He, D.Z., Cheng, J., Dallos, P., Madison, L.D. Audiol. Neurootol. (2002) [Pubmed]
  23. En block C-terminal charge cluster reversals in prestin (SLC26A5): effects on voltage-dependent electromechanical activity. Bai, J.P., Navaratnam, D., Samaranayake, H., Santos-Sacchi, J. Neurosci. Lett. (2006) [Pubmed]
  24. Conference report: functional magnetic resonance imaging for beginners--a review of the fMRI experience IV, 13-14 May 2002, Natcher Conference Center, National Institutes of Health, Bethesda, MD. Caggiano, D., Joffily, M. ScientificWorldJournal (2002) [Pubmed]
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