pendrin

Summary

Gene Symbol: pendrin
Description: solute carrier family 26, member 4
Alias: Pds, pendrin, pendrin, sodium-independent chloride/iodide transporter
Species: mouse
Products:     pendrin

Top Publications

  1. Verlander J, Hassell K, Royaux I, Glapion D, Wang M, Everett L, et al. Deoxycorticosterone upregulates PDS (Slc26a4) in mouse kidney: role of pendrin in mineralocorticoid-induced hypertension. Hypertension. 2003;42:356-62 pubmed
    b>Pendrin is an anion exchanger expressed along the apical plasma membrane and apical cytoplasmic vesicles of type B and of non-A, non-B intercalated cells of the distal convoluted tubule, connecting tubule, and cortical collecting duct...
  2. Hafner P, Grimaldi R, Capuano P, Capasso G, Wagner C. Pendrin in the mouse kidney is primarily regulated by Cl- excretion but also by systemic metabolic acidosis. Am J Physiol Cell Physiol. 2008;295:C1658-67 pubmed publisher
    The Cl(-)/anion exchanger pendrin (SLC26A4) is expressed on the apical side of renal non-type A intercalated cells. The abundance of pendrin is reduced during metabolic acidosis induced by oral NH(4)Cl loading...
  3. Choi B, Kim H, Ito T, Lee K, Li X, Monahan K, et al. Mouse model of enlarged vestibular aqueducts defines temporal requirement of Slc26a4 expression for hearing acquisition. J Clin Invest. 2011;121:4516-25 pubmed publisher
    ..SLC26A4 encodes pendrin, an anion-base exchanger expressed in inner ear epithelial cells that secretes HCO3- into endolymph...
  4. Everett L, Morsli H, Wu D, Green E. Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear. Proc Natl Acad Sci U S A. 1999;96:9727-32 pubmed
    ..we sought to investigate in greater detail the expression of the gene and the function of its encoded protein (pendrin)...
  5. Everett L, Belyantseva I, Noben Trauth K, Cantos R, Chen A, Thakkar S, et al. Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome. Hum Mol Genet. 2001;10:153-61 pubmed
    ..on defining the role of PDS in deafness and PS as well as elucidating the function of the PDS-encoded protein (pendrin)...
  6. Yang T, Vidarsson H, Rodrigo Blomqvist S, Rosengren S, Enerback S, Smith R. Transcriptional control of SLC26A4 is involved in Pendred syndrome and nonsyndromic enlargement of vestibular aqueduct (DFNB4). Am J Hum Genet. 2007;80:1055-63 pubmed
    ..These results support a novel dosage-dependent model for the molecular pathogenesis of PS and nonsyndromic EVA that involves SLC26A4 and its transcriptional regulatory machinery...
  7. Yang T, Gurrola J, Wu H, Chiu S, Wangemann P, Snyder P, et al. Mutations of KCNJ10 together with mutations of SLC26A4 cause digenic nonsyndromic hearing loss associated with enlarged vestibular aqueduct syndrome. Am J Hum Genet. 2009;84:651-7 pubmed publisher
    ..Our results link KCNJ10 mutations with EVA/PS and provide further support for the model of EVA/PS as a multigenic complex disease. ..
  8. Wangemann P, Kim H, Billings S, Nakaya K, Li X, Singh R, et al. Developmental delays consistent with cochlear hypothyroidism contribute to failure to develop hearing in mice lacking Slc26a4/pendrin expression. Am J Physiol Renal Physiol. 2009;297:F1435-47 pubmed publisher
    ..SLC26A4 encodes pendrin, an anion exchanger located in the cochlea, thyroid, and kidney...
  9. Vallet M, Picard N, Loffing Cueni D, Fysekidis M, Bloch Faure M, Deschenes G, et al. Pendrin regulation in mouse kidney primarily is chloride-dependent. J Am Soc Nephrol. 2006;17:2153-63 pubmed
    Recent studies indicate that pendrin, an apical Cl-/HCO3- exchanger, mediates chloride reabsorption in the connecting tubule and the cortical collecting duct and therefore is involved in extracellular fluid volume regulation...
  10. Dou H, Xu J, Wang Z, Smith A, Soleimani M, Karet F, et al. Co-expression of pendrin, vacuolar H+-ATPase alpha4-subunit and carbonic anhydrase II in epithelial cells of the murine endolymphatic sac. J Histochem Cytochem. 2004;52:1377-84 pubmed
    ..patterns of acid-base regulators, including vacuolar (v)H+-ATPase (proton pump), carbonic anhydrase (CA) II, and pendrin in the murine ES epithelium by immunohistochemistry (IHC) and compared their expression patterns by double ..

Detail Information

Publications62

  1. Verlander J, Hassell K, Royaux I, Glapion D, Wang M, Everett L, et al. Deoxycorticosterone upregulates PDS (Slc26a4) in mouse kidney: role of pendrin in mineralocorticoid-induced hypertension. Hypertension. 2003;42:356-62 pubmed
    b>Pendrin is an anion exchanger expressed along the apical plasma membrane and apical cytoplasmic vesicles of type B and of non-A, non-B intercalated cells of the distal convoluted tubule, connecting tubule, and cortical collecting duct...
  2. Hafner P, Grimaldi R, Capuano P, Capasso G, Wagner C. Pendrin in the mouse kidney is primarily regulated by Cl- excretion but also by systemic metabolic acidosis. Am J Physiol Cell Physiol. 2008;295:C1658-67 pubmed publisher
    The Cl(-)/anion exchanger pendrin (SLC26A4) is expressed on the apical side of renal non-type A intercalated cells. The abundance of pendrin is reduced during metabolic acidosis induced by oral NH(4)Cl loading...
  3. Choi B, Kim H, Ito T, Lee K, Li X, Monahan K, et al. Mouse model of enlarged vestibular aqueducts defines temporal requirement of Slc26a4 expression for hearing acquisition. J Clin Invest. 2011;121:4516-25 pubmed publisher
    ..SLC26A4 encodes pendrin, an anion-base exchanger expressed in inner ear epithelial cells that secretes HCO3- into endolymph...
  4. Everett L, Morsli H, Wu D, Green E. Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear. Proc Natl Acad Sci U S A. 1999;96:9727-32 pubmed
    ..we sought to investigate in greater detail the expression of the gene and the function of its encoded protein (pendrin)...
  5. Everett L, Belyantseva I, Noben Trauth K, Cantos R, Chen A, Thakkar S, et al. Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome. Hum Mol Genet. 2001;10:153-61 pubmed
    ..on defining the role of PDS in deafness and PS as well as elucidating the function of the PDS-encoded protein (pendrin)...
  6. Yang T, Vidarsson H, Rodrigo Blomqvist S, Rosengren S, Enerback S, Smith R. Transcriptional control of SLC26A4 is involved in Pendred syndrome and nonsyndromic enlargement of vestibular aqueduct (DFNB4). Am J Hum Genet. 2007;80:1055-63 pubmed
    ..These results support a novel dosage-dependent model for the molecular pathogenesis of PS and nonsyndromic EVA that involves SLC26A4 and its transcriptional regulatory machinery...
  7. Yang T, Gurrola J, Wu H, Chiu S, Wangemann P, Snyder P, et al. Mutations of KCNJ10 together with mutations of SLC26A4 cause digenic nonsyndromic hearing loss associated with enlarged vestibular aqueduct syndrome. Am J Hum Genet. 2009;84:651-7 pubmed publisher
    ..Our results link KCNJ10 mutations with EVA/PS and provide further support for the model of EVA/PS as a multigenic complex disease. ..
  8. Wangemann P, Kim H, Billings S, Nakaya K, Li X, Singh R, et al. Developmental delays consistent with cochlear hypothyroidism contribute to failure to develop hearing in mice lacking Slc26a4/pendrin expression. Am J Physiol Renal Physiol. 2009;297:F1435-47 pubmed publisher
    ..SLC26A4 encodes pendrin, an anion exchanger located in the cochlea, thyroid, and kidney...
  9. Vallet M, Picard N, Loffing Cueni D, Fysekidis M, Bloch Faure M, Deschenes G, et al. Pendrin regulation in mouse kidney primarily is chloride-dependent. J Am Soc Nephrol. 2006;17:2153-63 pubmed
    Recent studies indicate that pendrin, an apical Cl-/HCO3- exchanger, mediates chloride reabsorption in the connecting tubule and the cortical collecting duct and therefore is involved in extracellular fluid volume regulation...
  10. Dou H, Xu J, Wang Z, Smith A, Soleimani M, Karet F, et al. Co-expression of pendrin, vacuolar H+-ATPase alpha4-subunit and carbonic anhydrase II in epithelial cells of the murine endolymphatic sac. J Histochem Cytochem. 2004;52:1377-84 pubmed
    ..patterns of acid-base regulators, including vacuolar (v)H+-ATPase (proton pump), carbonic anhydrase (CA) II, and pendrin in the murine ES epithelium by immunohistochemistry (IHC) and compared their expression patterns by double ..
  11. Wangemann P, Itza E, Albrecht B, Wu T, Jabba S, Maganti R, et al. Loss of KCNJ10 protein expression abolishes endocochlear potential and causes deafness in Pendred syndrome mouse model. BMC Med. 2004;2:30 pubmed
    ..disorder characterized by congenital deafness and goiter, is caused by mutations of SLC26A4, which codes for pendrin. We investigated the relationship between pendrin and deafness using mice that have (Slc26a4+/+) or lack a ..
  12. Hulander M, Kiernan A, Blomqvist S, Carlsson P, Samuelsson E, Johansson B, et al. Lack of pendrin expression leads to deafness and expansion of the endolymphatic compartment in inner ears of Foxi1 null mutant mice. Development. 2003;130:2013-25 pubmed
    ..duct/sac epithelium shows a complete lack of the transcript encoding the chloride/iodide transporter pendrin. Based on this, we would like to suggest that Foxi1 is an upstream regulator of pendrin and that the phenotype ..
  13. Dror A, Politi Y, Shahin H, Lenz D, Dossena S, Nofziger C, et al. Calcium oxalate stone formation in the inner ear as a result of an Slc26a4 mutation. J Biol Chem. 2010;285:21724-35 pubmed publisher
    ..We show that a missense mutation within the Slc26a4 gene abolishes the transport activity of its encoded protein, pendrin. As a consequence, dramatic changes in mineral composition, size, and shape occur within the utricle and saccule ..
  14. Verlander J, Kim Y, Shin W, Pham T, Hassell K, Beierwaltes W, et al. Dietary Cl(-) restriction upregulates pendrin expression within the apical plasma membrane of type B intercalated cells. Am J Physiol Renal Physiol. 2006;291:F833-9 pubmed
    b>Pendrin, encoded by Slc26a4, is a Cl(-)/HCO(3)(-) exchanger expressed in the apical region of type B and non-A, non-B intercalated cells, which regulates renal NaCl excretion...
  15. Wangemann P, Nakaya K, Wu T, Maganti R, Itza E, Sanneman J, et al. Loss of cochlear HCO3- secretion causes deafness via endolymphatic acidification and inhibition of Ca2+ reabsorption in a Pendred syndrome mouse model. Am J Physiol Renal Physiol. 2007;292:F1345-53 pubmed
    ..childhood deafness and postpuberty goiter, is caused by mutations of SLC26A4, which codes for the anion exchanger pendrin. The goal of the present study was to determine how loss of pendrin leads to hair cell degeneration and deafness...
  16. Pech V, Pham T, Hong S, Weinstein A, Spencer K, Duke B, et al. Pendrin modulates ENaC function by changing luminal HCO3-. J Am Soc Nephrol. 2010;21:1928-41 pubmed publisher
    The epithelial Na(+) channel, ENaC, and the Cl(-)/HCO(3)(-) exchanger, pendrin, mediate NaCl absorption within the cortical collecting duct and the connecting tubule...
  17. Kim H, Wangemann P. Failure of fluid absorption in the endolymphatic sac initiates cochlear enlargement that leads to deafness in mice lacking pendrin expression. PLoS ONE. 2010;5:e14041 pubmed publisher
    ..Failure of fluid absorption in the endolymphatic sac due to lack of Slc26a4 expression appears to initiate cochlear enlargement in mice, and possibly humans, lacking functional Slc26a4 expression...
  18. Kim H, Wangemann P. Epithelial cell stretching and luminal acidification lead to a retarded development of stria vascularis and deafness in mice lacking pendrin. PLoS ONE. 2011;6:e17949 pubmed publisher
    Loss-of-function mutations of SLC26A4/pendrin are among the most prevalent causes of deafness...
  19. Wall S, Kim Y, Stanley L, Glapion D, Everett L, Green E, et al. NaCl restriction upregulates renal Slc26a4 through subcellular redistribution: role in Cl- conservation. Hypertension. 2004;44:982-7 pubmed
    Slc26a4 (Pds, pendrin) is an anion transporter expressed in the apical region of type B and non-A, non-B intercalated cells of the distal nephron...
  20. Kim Y, Kwon T, Frische S, Kim J, Tisher C, Madsen K, et al. Immunocytochemical localization of pendrin in intercalated cell subtypes in rat and mouse kidney. Am J Physiol Renal Physiol. 2002;283:F744-54 pubmed
    Recent studies have demonstrated that a novel anion exchanger, pendrin, is expressed in the apical domain of type B intercalated cells in the mammalian collecting duct...
  21. Li X, Zhou F, Marcus D, Wangemann P. Endolymphatic Na? and K? concentrations during cochlear growth and enlargement in mice lacking Slc26a4/pendrin. PLoS ONE. 2013;8:e65977 pubmed publisher
    ..The data suggest that the endolymphatic enlargement caused by a loss of Slc26a4 is a consequence of disrupted Na(+) transport...
  22. Kim Y, Pech V, Spencer K, Beierwaltes W, Everett L, Green E, et al. Reduced ENaC protein abundance contributes to the lower blood pressure observed in pendrin-null mice. Am J Physiol Renal Physiol. 2007;293:F1314-24 pubmed
    b>Pendrin (encoded by Pds, Slc26a4) is a Cl(-)/HCO(3)(-) exchanger expressed in the apical regions of type B and non-A, non-B intercalated cells of kidney and mediates renal Cl(-) absorption, particularly when upregulated...
  23. Lu Y, Wu C, Shen W, Yang T, Yeh T, Chen P, et al. Establishment of a knock-in mouse model with the SLC26A4 c.919-2A>G mutation and characterization of its pathology. PLoS ONE. 2011;6:e22150 pubmed publisher
    ..S408F mutation, albeit the severity of vestibular hair cell degeneration appeared different among the three mouse strains. ..
  24. Rebeh I, Yoshimi N, Hadj Kacem H, Yanohco S, Hammami B, Mnif M, et al. Two missense mutations in SLC26A4 gene: a molecular and functional study. Clin Genet. 2010;78:74-80 pubmed publisher
    Mutations in the SLC26A4 gene encoding pendrin, an anion transporter, are responsible for non-syndromic hearing loss (HL) (DFNB4) and Pendred syndrome (PS). PS is a genetic disorder that causes early HL and affects the thyroid gland...
  25. Shcheynikov N, Yang D, Wang Y, Zeng W, Karniski L, So I, et al. The Slc26a4 transporter functions as an electroneutral Cl-/I-/HCO3- exchanger: role of Slc26a4 and Slc26a6 in I- and HCO3- secretion and in regulation of CFTR in the parotid duct. J Physiol. 2008;586:3813-24 pubmed publisher
  26. Li X, Sanneman J, Harbidge D, Zhou F, Ito T, Nelson R, et al. SLC26A4 targeted to the endolymphatic sac rescues hearing and balance in Slc26a4 mutant mice. PLoS Genet. 2013;9:e1003641 pubmed publisher
    ..SLC26A4 encodes pendrin, an anion exchanger expressed in a variety of epithelial cells in the cochlea, the vestibular labyrinth and the ..
  27. Singh R, Wangemann P. Free radical stress-mediated loss of Kcnj10 protein expression in stria vascularis contributes to deafness in Pendred syndrome mouse model. Am J Physiol Renal Physiol. 2008;294:F139-48 pubmed
    Pendred syndrome is due to loss-of-function mutations of Slc26a4, which codes for the HCO(3)(-) transporter pendrin. Loss of pendrin causes deafness via a loss of the K(+) channel Kcnj10 in stria vascularis and consequent loss of the ..
  28. Nakaya K, Harbidge D, Wangemann P, Schultz B, Green E, Wall S, et al. Lack of pendrin HCO3- transport elevates vestibular endolymphatic [Ca2+] by inhibition of acid-sensitive TRPV5 and TRPV6 channels. Am J Physiol Renal Physiol. 2007;292:F1314-21 pubmed
    ..pH, [Ca(2+)], and transepithelial potential of the utricle were measured in Cl(-)/HCO(3)(-) exchanger pendrin (SLC26A4) knockout mice in vivo...
  29. Royaux I, Belyantseva I, Wu T, Kachar B, Everett L, Marcus D, et al. Localization and functional studies of pendrin in the mouse inner ear provide insight about the etiology of deafness in pendred syndrome. J Assoc Res Otolaryngol. 2003;4:394-404 pubmed
    ..studies of mouse cochlea and vestibular end-organ were performed to study the expression pattern of pendrin, the protein encoded by the Pendred syndrome gene (PDS), in the inner ear...
  30. Adler L, Efrati E, Zelikovic I. Molecular mechanisms of epithelial cell-specific expression and regulation of the human anion exchanger (pendrin) gene. Am J Physiol Cell Physiol. 2008;294:C1261-76 pubmed publisher
    b>Pendrin, a Cl(-)/anion exchanger encoded by the gene PDS, is highly expressed in the kidney, thyroid, and inner ear epithelia and is essential for bicarbonate secretion, iodide accumulation, and endolymph ion balance, respectively...
  31. Wall S, Hassell K, Royaux I, Green E, Chang J, Shipley G, et al. Localization of pendrin in mouse kidney. Am J Physiol Renal Physiol. 2003;284:F229-41 pubmed
    b>Pendrin is an anion exchanger expressed in type B intercalated cells of the cortical collecting duct (CCD). Whether pendrin localizes to other nephron segments with intercalated cells is unknown...
  32. Wagner C, Finberg K, Stehberger P, Lifton R, Giebisch G, Aronson P, et al. Regulation of the expression of the Cl-/anion exchanger pendrin in mouse kidney by acid-base status. Kidney Int. 2002;62:2109-17 pubmed
    b>Pendrin belongs to a superfamily of Cl-/anion exchangers and is expressed in the inner ear, the thyroid gland, and the kidney. In humans, mutations in pendrin cause Pendred syndrome characterized by sensorineural deafness and goiter...
  33. Sutliff R, Walp E, Kim Y, Walker L, El Ali A, Ma J, et al. Contractile force is enhanced in Aortas from pendrin null mice due to stimulation of angiotensin II-dependent signaling. PLoS ONE. 2014;9:e105101 pubmed publisher
    b>Pendrin is a Cl-/HCO3- exchanger expressed in the apical regions of renal intercalated cells. Following pendrin gene ablation, blood pressure falls, in part, from reduced renal NaCl absorption...
  34. Bronckers A, Guo J, Zandieh Doulabi B, Bervoets T, Lyaruu D, Li X, et al. Developmental expression of solute carrier family 26A member 4 (SLC26A4/pendrin) during amelogenesis in developing rodent teeth. Eur J Oral Sci. 2011;119 Suppl 1:185-92 pubmed publisher
    ..Solute carrier family 26A member 4 (SLC26A4, or pendrin) is an anion exchanger for chloride, bicarbonate, iodine, and formate...
  35. Blomqvist S, Vidarsson H, Fitzgerald S, Johansson B, Ollerstam A, Brown R, et al. Distal renal tubular acidosis in mice that lack the forkhead transcription factor Foxi1. J Clin Invest. 2004;113:1560-70 pubmed
    ..Thus, Foxi1(-/-) mice seem to develop dRTA due to altered cellular composition of the distal nephron epithelium, thereby denying this epithelium the proper gene expression pattern needed for maintaining adequate acid-base homeostasis. ..
  36. Pech V, Wall S, Nanami M, Bao H, Kim Y, Lazo Fernández Y, et al. Pendrin gene ablation alters ENaC subcellular distribution and open probability. Am J Physiol Renal Physiol. 2015;309:F154-63 pubmed publisher
    The present study explored whether the intercalated cell Cl(-)/HCO3(-) exchanger pendrin modulates epithelial Na(+) channel (ENaC) function by changing channel open probability and/or channel density...
  37. Knauf F, Yang C, Thomson R, Mentone S, Giebisch G, Aronson P. Identification of a chloride-formate exchanger expressed on the brush border membrane of renal proximal tubule cells. Proc Natl Acad Sci U S A. 2001;98:9425-30 pubmed
    ..Cl(-) reabsorption in the proximal tubule, we screened the expressed sequence tag database for homologs of pendrin, a transporter previously shown to mediate Cl(-)-formate exchange...
  38. Chambrey R, Kurth I, Peti Peterdi J, Houillier P, Purkerson J, Leviel F, et al. Renal intercalated cells are rather energized by a proton than a sodium pump. Proc Natl Acad Sci U S A. 2013;110:7928-33 pubmed publisher
    ..This concept is likely to apply to other animal cell types characterized by plasma membrane expression of the H(+) V-ATPase...
  39. Pelzl L, Pakladok T, Pathare G, Fakhri H, Michael D, Wagner C, et al. DOCA sensitive pendrin expression in kidney, heart, lung and thyroid tissues. Cell Physiol Biochem. 2012;30:1491-501 pubmed publisher
    b>Pendrin (SLC26A4), a transporter accomplishing anion exchange, is expressed in inner ear, thyroid gland, kidneys, lung, liver and heart...
  40. Soleimani M, Barone S, Xu J, Alshahrani S, Brooks M, McCormack F, et al. Prostaglandin-E2 Mediated Increase in Calcium and Phosphate Excretion in a Mouse Model of Distal Nephron Salt Wasting. PLoS ONE. 2016;11:e0159804 pubmed publisher
    ..b>Pendrin/NCC double KO (pendrin/NCC-dKO) mice display severe salt wasting under basal conditions and develop profound ..
  41. Choo D, Ward J, Reece A, Dou H, Lin Z, Greinwald J. Molecular mechanisms underlying inner ear patterning defects in kreisler mutants. Dev Biol. 2006;289:308-17 pubmed
    ..The data also identify Gbx2, Dlx5, Wnt2b and Otx2 as key otic genes ultimately affected by perturbation of the kr/mafB-hindbrain pathway. ..
  42. Eskalli Z, Achouri Y, Hahn S, Many M, Craps J, Refetoff S, et al. Overexpression of Interleukin-4 in the Thyroid of Transgenic Mice Upregulates the Expression of Duox1 and the Anion Transporter Pendrin. Thyroid. 2016;26:1499-1512 pubmed
    ..Upregulated expression of Duox1, Duoxa1, and the pendrin anion exchanger gene (Slc26a4) was detected...
  43. Jeong H, Jeon U, Koo B, Kim W, Im S, Shin J, et al. Inactivation of Notch signaling in the renal collecting duct causes nephrogenic diabetes insipidus in mice. J Clin Invest. 2009;119:3290-300 pubmed publisher
  44. Muñoz Espín D, Canamero M, Maraver A, Gomez Lopez G, Contreras J, Murillo Cuesta S, et al. Programmed cell senescence during mammalian embryonic development. Cell. 2013;155:1104-18 pubmed publisher
    ..We conclude that the role of developmentally programmed senescence is to promote tissue remodeling and propose that this is the evolutionary origin of damage-induced senescence. ..
  45. Patel Chamberlin M, Varasteh Kia M, Xu J, Barone S, Zahedi K, Soleimani M. The Role of Epithelial Sodium Channel ENaC and the Apical Cl-/HCO3- Exchanger Pendrin in Compensatory Salt Reabsorption in the Setting of Na-Cl Cotransporter (NCC) Inactivation. PLoS ONE. 2016;11:e0150918 pubmed publisher
    ..We hypothesized that ENaC and pendrin play important roles in compensatory salt absorption in the setting of NCC inactivation, and their inhibition and/..
  46. Everett L. New insights into the role of pendrin (SLC26A4) in inner ear fluid homeostasis. Novartis Found Symp. 2006;273:213-25; discussion 225-30, 261-4 pubmed
    ..that an anion transporter was responsible for these clinical features soon highlighted a potential role for pendrin in thyroid hormone biosynthesis. The role of pendrin in deafness, however, remained unclear...
  47. Thumová M, Pech V, FROEHLICH O, Agazatian D, Wang X, Verlander J, et al. Pendrin protein abundance in the kidney is regulated by nitric oxide and cAMP. Am J Physiol Renal Physiol. 2012;303:F812-20 pubmed publisher
    b>Pendrin is a Cl(-)/HCO(3)(-) exchanger, expressed in the apical regions of some intercalated cell subtypes, and is critical in the pressor response to angiotensin II...
  48. Soleimani M, Barone S, Xu J, Shull G, Siddiqui F, Zahedi K, et al. Double knockout of pendrin and Na-Cl cotransporter (NCC) causes severe salt wasting, volume depletion, and renal failure. Proc Natl Acad Sci U S A. 2012;109:13368-73 pubmed publisher
    ..is the target of inhibition by thiazides, is located in close proximity to the chloride-absorbing transporter pendrin in the kidney distal nephron...
  49. Azroyan A, Laghmani K, Crambert G, Mordasini D, Doucet A, EDWARDS A. Regulation of pendrin by pH: dependence on glycosylation. Biochem J. 2011;434:61-72 pubmed publisher
    Mutations in the anion exchanger pendrin are responsible for Pendred syndrome, an autosomal recessive disease characterized by deafness and goitre...
  50. Trepiccione F, Soukaseum C, Baudrie V, Kumai Y, Teulon J, Villoutreix B, et al. Acute genetic ablation of pendrin lowers blood pressure in mice. Nephrol Dial Transplant. 2017;32:1137-1145 pubmed publisher
    b>Pendrin, the chloride/bicarbonate exchanger of β-intercalated cells of the renal connecting tubule and the collecting duct, plays a key role in NaCl reabsorption by the distal nephron...
  51. Ito T, Nishio A, Wangemann P, Griffith A. Progressive irreversible hearing loss is caused by stria vascularis degeneration in an Slc26a4-insufficient mouse model of large vestibular aqueduct syndrome. Neuroscience. 2015;310:188-97 pubmed publisher
    ..This model of primary strial atrophy may be used to explore the mechanisms of progressive hearing loss due to strial dysfunction. ..
  52. Alshahrani S, Rapoport R, Zahedi K, Jiang M, Nieman M, Barone S, et al. The non-diuretic hypotensive effects of thiazides are enhanced during volume depletion states. PLoS ONE. 2017;12:e0181376 pubmed publisher
    ..NCC KO on a salt restricted diet but not with normal diet; 2) in volume depleted but not in volume resuscitated pendrin/NCC dKO mice; the BP reduction occurs without any enhancement in salt excretion or reduction in cardiac output...
  53. Wangemann P. Mouse models for pendrin-associated loss of cochlear and vestibular function. Cell Physiol Biochem. 2013;32:157-65 pubmed publisher
    The human gene SLC26A4 and the mouse ortholog Slc26a4 code for the protein pendrin, which is an anion exchanger expressed in apical membranes of selected epithelia...
  54. Barone S, Amlal H, Xu J, Soleimani M. Deletion of the Cl-/HCO3- exchanger pendrin downregulates calcium-absorbing proteins in the kidney and causes calcium wasting. Nephrol Dial Transplant. 2012;27:1368-79 pubmed publisher
    The epithelial calcium channel (ECaC) (TRPV5) and the Cl-/HCO3- exchanger pendrin (SLC26A4) are expressed on the apical membrane of tubular cells in the distal nephron and play essential roles in calcium re-absorption and bicarbonate ..
  55. Burton Q, Cole L, Mulheisen M, Chang W, Wu D. The role of Pax2 in mouse inner ear development. Dev Biol. 2004;272:161-75 pubmed
  56. Nakagami Y, Favoreto S, Zhen G, Park S, Nguyenvu L, Kuperman D, et al. The epithelial anion transporter pendrin is induced by allergy and rhinovirus infection, regulates airway surface liquid, and increases airway reactivity and inflammation in an asthma model. J Immunol. 2008;181:2203-10 pubmed
    ..We previously reported that expression of the epithelial cell anion transporter pendrin is markedly increased in response to IL-13...
  57. Kim B, Kim J, Kim H, Bok J, Namkung W, Choi J, et al. Developmental changes of ENaC expression and function in the inner ear of pendrin knock-out mice as a perspective on the development of endolymphatic hydrops. PLoS ONE. 2014;9:e95730 pubmed publisher
    b>Pendrin mutations cause enlarged vestibular aqueducts and various degrees of sensorineural hearing loss...
  58. Raft S, Andrade L, Shao D, Akiyama H, Henkemeyer M, Wu D. Ephrin-B2 governs morphogenesis of endolymphatic sac and duct epithelia in the mouse inner ear. Dev Biol. 2014;390:51-67 pubmed publisher
    ..We propose that developmental dysplasias described here are a gene dose-sensitive cause of the vestibular dysfunction observed in EphB-Efnb2 signaling-deficient mice. ..
  59. Procino G, Milano S, Tamma G, Dossena S, Barbieri C, Nicoletti M, et al. Co-regulated pendrin and aquaporin 5 expression and trafficking in Type-B intercalated cells under potassium depletion. Cell Physiol Biochem. 2013;32:184-99 pubmed publisher
    ..reported that aquaporin 5 (AQP5), a water channel never identified in the kidney before, co-localizes with pendrin at the apical membrane of type-B intercalated cells in the kidney cortex...
  60. Nishio A, Ito T, Cheng H, Fitzgerald T, Wangemann P, Griffith A. Slc26a4 expression prevents fluctuation of hearing in a mouse model of large vestibular aqueduct syndrome. Neuroscience. 2016;329:74-82 pubmed publisher
    ..SLC26A4 encodes a transmembrane anion exchanger called pendrin expressed in nonsensory epithelial cells of the lateral wall of cochlea, vestibular organs and endolymphatic sac...
  61. Ito T, Li X, Kurima K, Choi B, Wangemann P, Griffith A. Slc26a4-insufficiency causes fluctuating hearing loss and stria vascularis dysfunction. Neurobiol Dis. 2014;66:53-65 pubmed publisher
    ..Our strategy to generate a hypomorphic mouse model utilizing the tet-on system will be applicable to other diseases in which a hypomorphic allele is needed to model the human phenotype. ..
  62. Sun X, Soleimani M, Petrovic S. Decreased expression of Slc26a4 (Pendrin) and Slc26a7 in the kidneys of carbonic anhydrase II-deficient mice. Cell Physiol Biochem. 2008;21:95-108 pubmed publisher
    ..Bicarbonate secretion is mediated via Pendrin (Slc26a4), which is expressed on the apical membrane of B-ICs and nonA-nonB ICs in the cortical collecting ducts (..