Everett LA, Glaser B, Beck JC, Idol JR, Buchs A, Heyman M, Adawi F, Hazani E, Nassir E, Baxevanis AD, Sheffield VC, Green ED. Pendred syndrome is caused by mutations in a putative sulphate transporter gene (PDS). Nat Genet. 1997;17:411–22.
Article
CAS
PubMed
Google Scholar
Wangemann P, Nakaya K, Wu T, Maganti RJ, Itza EM, Sanneman JD, Harbidge DG, Billings S, Marcus DC. 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.
Article
CAS
PubMed
PubMed Central
Google Scholar
Karet FE, Finberg KE, Nelson RD, Nayir A, Mocan H, Sanjad SA, Rodriguez-Soriano J, Santos F, Cremers CW, Di Pietro A, Hoffbrand BI, Winiarski J, Bakkaloglu A, Ozen S, Dusunsel R, Goodyer P, Hulton SA, Wu DK, Skvorak AB, Morton CC, Cunningham MJ, Jha V, Lifton RP. Mutations in the gene encoding B1 subunit of H+-ATPase cause renal tubular acidosis with sensorineural deafness. Nat Genet. 1999;21:84–90.
Article
CAS
PubMed
Google Scholar
Stover EH, Borthwick KJ, Bavalia C, Eady N, Fritz DM, Rungroj N, Giersch AB, Morton CC, Axon PR, Akil I, Al-Sabban EA, Baguley DM, Bianca S, Bakkaloglu A, Bircan Z, Chauveau D, Clermont MJ, Guala A, Hulton SA, Kroes H, Li VG, Mir S, Mocan H, Nayir A, Ozen S, Rodriguez-Soriano J, Sanjad SA, Tasic V, Taylor CM, Topaloglu R, Smith AN, Karet FE. Novel ATP6V1B1 and ATP6V0A4 mutations in autosomal recessive distal renal tubular acidosis with new evidence for hearing loss. J Med Genet. 2002;39:796–803.
Article
CAS
PubMed
PubMed Central
Google Scholar
Ikeda K, Morizono T. The preparation of acetic acid for use in otic drops and its effect on endocochlear potential and pH in inner ear fluid. Am J Otolaryngol. 1989;10:382–5.
Article
CAS
PubMed
Google Scholar
Tanaka F, Whitworth CA, Rybak LP. Influence of pH on the ototoxicity of cisplatin: a round window application study. Hear Res. 2003;177:21–31.
Article
CAS
PubMed
Google Scholar
Stankovic KM, Brown D, Alper SL, Adams JC. Localization of pH regulating proteins H+ATPase and Cl−/HCO3
− exchanger in the guinea pig inner ear. Hear Res. 1997;114:21–34.
Article
CAS
PubMed
Google Scholar
Shibata T, Hibino H, Doi K, Suzuki T, Hisa Y, Kurachi Y. Gastric type H+, K + -ATPase in the cochlear lateral wall is critically involved in formation of the endocochlear potential. Am J Physiol Cell Physiol. 2006;291:C1038–48.
Article
CAS
PubMed
Google Scholar
Bond BR, Ng LL, Schulte BA. Identification of mRNA transcripts and immunohistochemical localization of Na/H exchanger isoforms in gerbil inner ear. Hear Res. 1998;123:1–9.
Article
CAS
PubMed
Google Scholar
Goto S, Oshima T, Ikeda K, Takasaka T. Expression and localization of the Na+-H+ exchanger in the guinea pig cochlea. Hear Res. 1999;128:89–96.
Article
CAS
PubMed
Google Scholar
Ikeda K, Sunose H, Takasaka T. Involvement of Na+-H+ exchange in intracellular pH recovery from acid load in the stria vascularis of the guinea-pig cochlea. Acta Otolaryngol (Stockh). 1994;114:162–6.
Article
CAS
Google Scholar
Wangemann P, Liu J, Shiga N. Vestibular dark cells contain the Na+/H+ exchanger NHE-1 in the basolateral membrane. Hear Res. 1996;94:94–106.
Article
CAS
PubMed
Google Scholar
Shimozono M, Scofield MA, Wangemann P. Functional evidence for a monocarboxylate transporter (MCT) in strial marginal cells and molecular evidence for MCT1 and MCT2 in stria vascularis. Hear Res. 1997;114:213–22.
Article
CAS
PubMed
Google Scholar
Kim HM, 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.
Article
CAS
PubMed
PubMed Central
Google Scholar
Marcus DC, Shipley AM. Potassium secretion by vestibular dark cell epithelium demonstrated by vibrating probe. Biophys J. 1994;66:1939–42.
Article
CAS
PubMed
PubMed Central
Google Scholar
Sunose H, Liu J, Shen Z, Marcus DC. cAMP increases K+ secretion via activation of apical IsK/KvLQT1 channels in strial marginal cells. Hear Res. 1997;114:107–16.
Article
CAS
PubMed
Google Scholar
Wangemann P, Liu J, Marcus DC. Ion transport mechanisms responsible for K+ secretion and the transepithelial voltage across marginal cells of stria vascularis in vitro. Hear Res. 1995;84:19–29.
Article
CAS
PubMed
Google Scholar
Thalmann R, Miyoshi T, Kusakari J, Ise I. Normal and abnormal energy metabolism of the inner ear. Otolaryngol Clin North Am. 1975;8:313–33.
CAS
PubMed
Google Scholar
DeCoursey TE. Voltage-gated proton channels: molecular biology, physiology, and pathophysiology of the H(V) family. Physiol Rev. 2013;93:599–652.
Article
CAS
PubMed
PubMed Central
Google Scholar
Wang X, Horisberger JD. A conformation of Na+-K+ pump is permeable to proton. Am J Physiol Cell Physiol. 1995;268:C590–5.
CAS
Google Scholar
Marcus DC, Thalmann R, Marcus NY. Respiratory quotient of stria vascularis of guinea pig in vitro. Arch Otorhinolaryngol. 1978;221:97–103.
Article
CAS
PubMed
Google Scholar
Marcus DC, Thalmann R, Marcus NY. Respiratory rate and ATP content of stria vascularis of guinea pig in vitro. Laryngoscope. 1978;88:1825–35.
Article
CAS
PubMed
Google Scholar
White PN, Thorne PR, Housley GD, Mockett B, Billett TE, Burnstock G. Quinacrine staining of marginal cells in the stria vascularis of the guinea-pig cochlea: a possible source of extracellular ATP? Hear Res. 1995;90:97–105.
Article
CAS
PubMed
Google Scholar
Marcus DC, Marcus NY, Greger R. Sidedness of action of loop diuretics and ouabain on nonsensory cells of utricle: a micro-Ussing chamber for inner ear tissues. Hear Res. 1987;30:55–64.
Article
CAS
PubMed
Google Scholar
Marcus NY, Marcus DC. Potassium secretion by nonsensory region of gerbil utricle in vitro. Am J Physiol. 1987;253:F613–21.
CAS
PubMed
Google Scholar
Krishnamurthy H, Piscitelli CL, Gouaux E. Unlocking the molecular secrets of sodium-coupled transporters. Nature. 2009;459:347–55.
Article
CAS
PubMed
Google Scholar
Shen Z, Liu J, Marcus DC, Shiga N, Wangemann P. DIDS increases K+ secretion through an IsK channel in apical membrane of vestibular dark cell epithelium of gerbil. J Membr Biol. 1995;146:283–91.
Article
CAS
PubMed
Google Scholar
Parker MD, Boron WF. The divergence, actions, roles, and relatives of sodium-coupled bicarbonate transporters. Physiol Rev. 2013;93:803–959.
Article
CAS
PubMed
PubMed Central
Google Scholar
Masereel B, Pochet L, Laeckmann D. An overview of inhibitors of Na(+)/H(+) exchanger. Eur J Med Chem. 2003;38:547–54.
Article
CAS
PubMed
Google Scholar
Pondugula SR, Kampalli SB, Wu T, De Lisle RC, Raveendran NN, Harbidge DG, Marcus DC. cAMP-stimulated Cl- secretion is increased by glucocorticoids and inhibited by bumetanide in semicircular canal duct epithelium. BMC Physiol. 2013;13:6.
Article
CAS
PubMed
PubMed Central
Google Scholar
Wangemann P, Wittner M, Di SA, Englert HC, Lang HJ, Schlatter E, Greger R. Cl(-)-channel blockers in the thick ascending limb of the loop of Henle. Structure activity relationship. Pflugers Arch. 1986;407 Suppl 2:S128–41.
Article
CAS
PubMed
Google Scholar
Tse CM, Levine SA, Yun CH, Montrose MH, Little PJ, Pouyssegur J, Donowitz M. Cloning and expression of a rabbit cDNA encoding a serum-activated ethylisopropylamiloride-resistant epithelial Na+/H+ exchanger isoform (NHE-2). J Biol Chem. 1993;268:11917–24.
CAS
PubMed
Google Scholar
Soleimani M, Singh G, Bizal GL, Gullans SR, McAteer JA. Na+/H+ exchanger isoforms NHE-2 and NHE-1 in inner medullary collecting duct cells. Expression, functional localization, and differential regulation. J Biol Chem. 1994;269:27973–8.
CAS
PubMed
Google Scholar
Hill JK, Brett CL, Chyou A, Kallay LM, Sakaguchi M, Rao R, Gillespie PG. Vestibular hair bundles control pH with (Na+, K+)/H+ exchangers NHE6 and NHE9. J Neurosci. 2006;26:9944–55.
Article
CAS
PubMed
Google Scholar
Murphy E, Eisner DA. Regulation of intracellular and mitochondrial sodium in health and disease. Circ Res. 2009;104:292–303.
Article
CAS
PubMed
PubMed Central
Google Scholar
Marcus DC, Marcus NY, Thalmann R. Changes in cation contents of stria vascularis with ouabain and potassium-free perfusion. Hear Res. 1981;4:149–60.
Article
CAS
PubMed
Google Scholar
Kuijpers W, Bonting SL. Studies on (Na + -K+)-activated ATPase. XXIV. Localization and properties of ATPase in the inner ear of the guinea pig. Biochim Biophys Acta. 1969;173:477–85.
Article
CAS
PubMed
Google Scholar
Yoo JC, Kim HY, Han KH, Oh SH, Chang SO, Marcus DC, Lee JH. Na(+) absorption by Claudius’ cells is regulated by purinergic signaling in the cochlea. Acta Otolaryngol. 2012;132 Suppl 1:S103–8.
Article
CAS
PubMed
Google Scholar
Kim SH, Marcus DC. Regulation of sodium transport in the inner ear. Hear Res. 2011;280:21–9.
Article
CAS
PubMed
PubMed Central
Google Scholar
Yamazaki M, Kim KX, Marcus DC. Sodium selectivity of Reissner’s membrane epithelial cells. BMC Physiol. 2011;11:4.
Article
CAS
PubMed
PubMed Central
Google Scholar
Kim SH, Kim KX, Raveendran NN, Wu T, Pondugula SR, Marcus DC. Regulation of ENaC-mediated sodium transport by glucocorticoids in Reissner’s membrane epithelium. Am J Physiol Cell Physiol. 2009;296:C544–57.
Article
CAS
PubMed
PubMed Central
Google Scholar
Lee JH, Marcus DC. Endolymphatic sodium homeostasis by Reissner’s membrane. Neuroscience. 2003;119:3–8.
Article
CAS
PubMed
Google Scholar
Marcus DC, Chiba T. K+ and Na+ absorption by outer sulcus epithelial cells. Hear Res. 1999;134:48–56.
Article
CAS
PubMed
Google Scholar
Yamauchi D, Raveendran NN, Pondugula SR, Kampalli SB, Sanneman JD, Harbidge DG, Marcus DC. Vitamin D upregulates expression of ECaC1 mRNA in semicircular canal. Biochem Biophys Res Commun. 2005;331:1353–7.
Article
CAS
PubMed
Google Scholar
Yamauchi D, Nakaya K, Raveendran NN, Harbidge DG, Singh R, Wangemann P, Marcus DC. Expression of epithelial calcium transport system in rat cochlea and vestibular labyrinth. BMC Physiol. 2010;10:1.
Article
PubMed
PubMed Central
Google Scholar
Wood JD, Muchinsky SJ, Filoteo AG, Penniston JT, Tempel BL. Low endolymph calcium concentrations in deafwaddler2J mice suggest that PMCA2 contributes to endolymph calcium maintenance. J Assoc Res Otolaryngol. 2004;5:99–110.
Article
PubMed
PubMed Central
Google Scholar
Scherer EQ, Yang J, Canis M, Reimann K, Ivanov K, Diehl CD, Backx PH, Wier WG, Strieth S, Wangemann P, Voigtlaender-Bolz J, Lidington D, Bolz SS. Tumor necrosis factor-alpha enhances microvascular tone and reduces blood flow in the cochlea via enhanced sphingosine-1-phosphate signaling. Stroke. 2010;41:2618–24.
Article
CAS
PubMed
PubMed Central
Google Scholar
Sellick PM, Johnstone BM. Changes in cochlear endolymph Na+ concentration measured with Na+ specific microelectrodes. Pflugers Arch. 1972;336:11–20.
Article
CAS
PubMed
Google Scholar
Wangemann P, Itza EM, Albrecht B, Wu T, Jabba SV, Maganti RJ, Lee JH, Everett LA, Wall SM, Royaux IE, Green ED, Marcus DC. Loss of KCNJ10 protein expression abolishes endocochlear potential and causes deafness in Pendred syndrome mouse model. BMC Med. 2004;2:30.
Article
PubMed
PubMed Central
Google Scholar
Codina J, DuBose Jr TD. Molecular regulation and physiology of the H+, K+ -ATPases in kidney. Semin Nephrol. 2006;26:345–51.
Article
CAS
PubMed
Google Scholar
Briving C, Andersson BM, Nordberg P, Wallmark B. Inhibition of gastric H+/K+-ATPase by substituted imidazo[1,2-a]pyridines. Biochim Biophys Acta. 1988;946:185–92.
Article
CAS
PubMed
Google Scholar
Wallmark B, Briving C, Fryklund J, Munson K, Jackson R, Mendlein J, Rabon E, Sachs G. Inhibition of gastric H+, K + -ATPase and acid secretion by SCH 28080, a substituted pyridyl(1,2a)imidazole. J Biol Chem. 1987;262:2077–84.
CAS
PubMed
Google Scholar
Unsold B, Kerst G, Brousos H, Hubner M, Schreiber R, Nitschke R, Greger R, Bleich M. KCNE1 reverses the response of the human K+ channel KCNQ1 to cytosolic pH changes and alters its pharmacology and sensitivity to temperature. Pflugers Arch. 2000;441:368–78.
Article
CAS
PubMed
Google Scholar
Xu H, Cui N, Yang Z, Qu Z, Jiang C. Modulation of kir4.1 and kir5.1 by hypercapnia and intracellular acidosis. J Physiol Lond. 2000;524(Pt 3):725–35.
Article
CAS
PubMed
PubMed Central
Google Scholar