Failure of Fluid Absorption in the Endolymphatic Sac Initiates Cochlear Enlargement that Leads to Deafness in Mice Lacking Pendrin Expression

scientific article published on November 17, 2010

Failure of Fluid Absorption in the Endolymphatic Sac Initiates Cochlear Enlargement that Leads to Deafness in Mice Lacking Pendrin Expression is …
instance of (P31):
scholarly articleQ13442814

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P819ADS bibcode2010PLoSO...514041K
P356DOI10.1371/JOURNAL.PONE.0014041
P953full work available at URLhttps://europepmc.org/articles/PMC2984494
https://europepmc.org/articles/PMC2984494?pdf=render
https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0014041&type=printable
https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/21103348/?tool=EBI
https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/21103348/pdf/?tool=EBI
P932PMC publication ID2984494
P698PubMed publication ID21103348
P5875ResearchGate publication ID49631872

P50authorPhiline WangemannQ124360509
P2093author name stringHyoung-Mi Kim
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Loss of KCNJ10 protein expression abolishes endocochlear potential and causes deafness in Pendred syndrome mouse modelQ24805411
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Co-expression of pendrin, vacuolar H+-ATPase alpha4-subunit and carbonic anhydrase II in epithelial cells of the murine endolymphatic sacQ28509455
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Developmental acquisition of sensory transduction in hair cells of the mouse inner earQ30311224
Free radical stress-mediated loss of Kcnj10 protein expression in stria vascularis contributes to deafness in Pendred syndrome mouse modelQ30413304
Tonotopic gradient in the developmental acquisition of sensory transduction in outer hair cells of the mouse cochleaQ30435999
Localization and functional studies of pendrin in the mouse inner ear provide insight about the etiology of deafness in pendred syndromeQ30473447
Comprehensive molecular etiology analysis of nonsyndromic hearing impairment from typical areas in ChinaQ30486353
Clinical characteristics and genotype-phenotype correlation of hearing loss patients with SLC26A4 mutationsQ50455675
Long-term follow-up of hearing loss in children and young adults with enlarged vestibular aqueducts: relationship to radiologic findings and Pendred syndrome diagnosisQ50460979
SLC26A4 gene is frequently involved in nonsyndromic hearing impairment with enlarged vestibular aqueduct in Caucasian populations.Q50466201
Neuro-otological findings in Pendred syndrome.Q50484453
Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome.Q50493197
ASSOCIATION OF CONGENITAL DEAFNESS WITH GOITRE (PENDRED'S SYNDROME) A STUDY OF 207 FAMILIES.Q50678597
Developmental and cellular expression pattern of epithelial sodium channel alpha, beta and gamma subunits in the inner ear of the rat.Q52140661
Development of monovalent ions in the endolymph in mouse cochlea.Q52165666
Cation transport in the ampulla of the semicircular canal and in the endolymphatic sac.Q53882005
Quantification of the "in vitro" development of the mouse embryo inner earQ54504407
Na,K-ATPase subunit isoform expression in the guinea pig endolymphatic sacQ72831274
Evidence for the presence of a Na+-H+ exchanger in the endolymphatic sac epithelium of guinea-pigsQ74567921
A protective role for type 3 deiodinase, a thyroid hormone-inactivating enzyme, in cochlear development and auditory functionQ30486616
Loss of cochlear HCO3- secretion causes deafness via endolymphatic acidification and inhibition of Ca2+ reabsorption in a Pendred syndrome mouse modelQ30494661
An Experimental Approach to the Understanding and Treatment of Hereditary Syndromes with Congenital Deafness and HypothyroidismQ33585723
Prevalence and evolutionary origins of the del(GJB6-D13S1830) mutation in the DFNB1 locus in hearing-impaired subjects: a multicenter studyQ33905363
Pendred syndrome--100 years of underascertainment?Q34439749
Effect of locally applied drugs on the endolymphatic sac potentialQ34746805
Type 2 iodothyronine deiodinase expression in the cochlea before the onset of hearingQ35016845
Origins and frequencies of SLC26A4 (PDS) mutations in east and south Asians: global implications for the epidemiology of deafnessQ35442102
Energization of sodium absorption by the H(+)-ATPase pump in mitochondria-rich cells of frog skinQ35663639
Supporting sensory transduction: cochlear fluid homeostasis and the endocochlear potentialQ36542382
Lack of pendrin HCO3- transport elevates vestibular endolymphatic [Ca2+] by inhibition of acid-sensitive TRPV5 and TRPV6 channelsQ36835443
Hypo-functional SLC26A4 variants associated with nonsyndromic hearing loss and enlargement of the vestibular aqueduct: genotype-phenotype correlation or coincidental polymorphisms?Q37144696
Developmental delays consistent with cochlear hypothyroidism contribute to failure to develop hearing in mice lacking Slc26a4/pendrin expressionQ37430770
Interferon-gamma suppresses Na+ -H+ exchanger in cultured human endolymphatic sac epithelial cells.Q38494580
Hypothyroidism prevents developmental neuronal loss during auditory organ developmentQ42438800
Experimental blockage of the endolymphatic duct and sac and its effect on the inner ear of the guinea pig. A study on endolymphatic hydropsQ46161994
Localization of pH regulating proteins H+ATPase and Cl-/HCO3- exchanger in the guinea pig inner ear.Q46190394
Intracellular and extracellular ion content of the endolymphatic sac.Q46219437
Low-amiloride-affinity Na+ channel in the epithelial cells isolated from the endolymphatic sac of guinea-pigsQ46709341
ULTRASTRUCTURAL ORGANIZATION OF THE EPITHELIAL LINING IN THE ENDOLYMPHATIC DUCT AND SAC IN THE GUINEA PIG.Q46711205
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 ductQ48747441
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P433issue11
P407language of work or nameEnglishQ1860
P921main subjectdeafnessQ12133
P304page(s)e14041
P577publication date2010-11-17
P1433published inPLOS OneQ564954
P1476titleFailure of fluid absorption in the endolymphatic sac initiates cochlear enlargement that leads to deafness in mice lacking pendrin expression
Failure of Fluid Absorption in the Endolymphatic Sac Initiates Cochlear Enlargement that Leads to Deafness in Mice Lacking Pendrin Expression
P478volume5

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