Literature DB >> 19815857

Potassium ion movement in the inner ear: insights from genetic disease and mouse models.

Anselm A Zdebik1, Philine Wangemann, Thomas J Jentsch.   

Abstract

Sensory transduction in the cochlea and vestibular labyrinth depends on fluid movements that deflect the hair bundles of mechanosensitive hair cells. Mechanosensitive transducer channels at the tip of the hair cell stereocilia allow K(+) to flow into cells. This unusual process relies on ionic gradients unique to the inner ear. Linking genes to deafness in humans and mice has been instrumental in identifying the ion transport machinery important for hearing and balance. Morphological analysis is difficult in patients, but mouse models have helped to investigate phenotypes at different developmental time points. This review focuses on cellular ion transport mechanisms in the stria vascularis that generate the major electrochemical gradients for sensory transduction.

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Year:  2009        PMID: 19815857      PMCID: PMC4415853          DOI: 10.1152/physiol.00018.2009

Source DB:  PubMed          Journal:  Physiology (Bethesda)        ISSN: 1548-9221


  75 in total

1.  mRNA encoding 'ClC-K1, a kidney Cl(-)- channel' is expressed in marginal cells of the stria vascularis of rat cochlea: its possible contribution to Cl(-) currents.

Authors:  M Ando; S Takeuchi
Journal:  Neurosci Lett       Date:  2000-04-28       Impact factor: 3.046

2.  Compartmentalization established by claudin-11-based tight junctions in stria vascularis is required for hearing through generation of endocochlear potential.

Authors:  Shin-ichiro Kitajiri; Tatsuo Miyamoto; Akihito Mineharu; Noriyuki Sonoda; Kyoko Furuse; Masaki Hata; Hiroyuki Sasaki; Yoshiaki Mori; Takahiro Kubota; Juichi Ito; Mikio Furuse; Shoichiro Tsukita
Journal:  J Cell Sci       Date:  2004-10-01       Impact factor: 5.285

3.  Coassembly of K(V)LQT1 and minK (IsK) proteins to form cardiac I(Ks) potassium channel.

Authors:  M C Sanguinetti; M E Curran; A Zou; J Shen; P S Spector; D L Atkinson; M T Keating
Journal:  Nature       Date:  1996-11-07       Impact factor: 49.962

Review 4.  Comparison of ion transport mechanisms between vestibular dark cells and strial marginal cells.

Authors:  P Wangemann
Journal:  Hear Res       Date:  1995-10       Impact factor: 3.208

5.  Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome.

Authors:  L A Everett; I A Belyantseva; K Noben-Trauth; R Cantos; A Chen; S I Thakkar; S L Hoogstraten-Miller; B Kachar; D K Wu; E D Green
Journal:  Hum Mol Genet       Date:  2001-01-15       Impact factor: 6.150

6.  Novel ATP6V1B1 and ATP6V0A4 mutations in autosomal recessive distal renal tubular acidosis with new evidence for hearing loss.

Authors:  E H Stover; K J Borthwick; C Bavalia; N Eady; D M Fritz; N Rungroj; A B S Giersch; C C Morton; P R Axon; I Akil; E A Al-Sabban; D M Baguley; S Bianca; A Bakkaloglu; Z Bircan; D Chauveau; M-J Clermont; A Guala; S A Hulton; H Kroes; G Li Volti; S Mir; H Mocan; A Nayir; S Ozen; J Rodriguez Soriano; S A Sanjad; V Tasic; C M Taylor; R Topaloglu; A N Smith; F E Karet
Journal:  J Med Genet       Date:  2002-11       Impact factor: 6.318

7.  KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.

Authors:  T Kharkovets; J P Hardelin; S Safieddine; M Schweizer; A El-Amraoui; C Petit; T J Jentsch
Journal:  Proc Natl Acad Sci U S A       Date:  2000-04-11       Impact factor: 11.205

8.  GJB2 gene mutations in childhood deafness.

Authors:  S Angeli; R Utrera; S Dib; E Chiossone; C Naranjo; O Henríquez; M Porta
Journal:  Acta Otolaryngol       Date:  2000-03       Impact factor: 1.494

9.  Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SeSAME syndrome) caused by mutations in KCNJ10.

Authors:  Ute I Scholl; Murim Choi; Tiewen Liu; Vincent T Ramaekers; Martin G Häusler; Joanne Grimmer; Sheldon W Tobe; Anita Farhi; Carol Nelson-Williams; Richard P Lifton
Journal:  Proc Natl Acad Sci U S A       Date:  2009-03-16       Impact factor: 11.205

10.  Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential.

Authors:  Barbara Teubner; Vincent Michel; Jörg Pesch; Jürgen Lautermann; Martine Cohen-Salmon; Goran Söhl; Klaus Jahnke; Elke Winterhager; Claus Herberhold; Jean-Pierre Hardelin; Christine Petit; Klaus Willecke
Journal:  Hum Mol Genet       Date:  2003-01-01       Impact factor: 6.150

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  76 in total

Review 1.  Gene expression profiling of the inner ear.

Authors:  Thomas Schimmang; Mark Maconochie
Journal:  J Anat       Date:  2015-09-25       Impact factor: 2.610

2.  Osmotic stabilization prevents cochlear synaptopathy after blast trauma.

Authors:  Jinkyung Kim; Anping Xia; Nicolas Grillet; Brian E Applegate; John S Oghalai
Journal:  Proc Natl Acad Sci U S A       Date:  2018-05-07       Impact factor: 11.205

3.  Computational model of a circulation current that controls electrochemical properties in the mammalian cochlea.

Authors:  Fumiaki Nin; Hiroshi Hibino; Shingo Murakami; Toshihiro Suzuki; Yasuo Hisa; Yoshihisa Kurachi
Journal:  Proc Natl Acad Sci U S A       Date:  2012-05-22       Impact factor: 11.205

4.  The mechanism underlying maintenance of the endocochlear potential by the K+ transport system in fibrocytes of the inner ear.

Authors:  Naoko Adachi; Takamasa Yoshida; Fumiaki Nin; Genki Ogata; Soichiro Yamaguchi; Toshihiro Suzuki; Sizuo Komune; Yasuo Hisa; Hiroshi Hibino; Yoshihisa Kurachi
Journal:  J Physiol       Date:  2013-07-08       Impact factor: 5.182

5.  Immunohistochemical localization of megalin and cubilin in the human inner ear.

Authors:  Seiji Hosokawa; Kumiko Hosokawa; Gail Ishiyama; Akira Ishiyama; Ivan A Lopez
Journal:  Brain Res       Date:  2018-09-12       Impact factor: 3.252

Review 6.  Regulation of sodium transport in the inner ear.

Authors:  Sung Huhn Kim; Daniel C Marcus
Journal:  Hear Res       Date:  2011-05-18       Impact factor: 3.208

Review 7.  Molecular aspects of structure, gating, and physiology of pH-sensitive background K2P and Kir K+-transport channels.

Authors:  Francisco V Sepúlveda; L Pablo Cid; Jacques Teulon; María Isabel Niemeyer
Journal:  Physiol Rev       Date:  2015-01       Impact factor: 37.312

Review 8.  Physiology and pathophysiology of SLC12A1/2 transporters.

Authors:  Nicolas Markadieu; Eric Delpire
Journal:  Pflugers Arch       Date:  2013-10-06       Impact factor: 3.657

9.  Immunological characterization of two types of ionocytes in the inner ear epithelium of Pacific Chub Mackerel (Scomber japonicus).

Authors:  Garfield T Kwan; Taylor R Smith; Martin Tresguerres
Journal:  J Comp Physiol B       Date:  2020-05-28       Impact factor: 2.200

10.  The human deafness-associated connexin 30 T5M mutation causes mild hearing loss and reduces biochemical coupling among cochlear non-sensory cells in knock-in mice.

Authors:  Melanie Schütz; Pietro Scimemi; Paromita Majumder; Romolo Daniele De Siati; Giulia Crispino; Laura Rodriguez; Mario Bortolozzi; Rosamaria Santarelli; Anke Seydel; Stephan Sonntag; Neil Ingham; Karen P Steel; Klaus Willecke; Fabio Mammano
Journal:  Hum Mol Genet       Date:  2010-09-21       Impact factor: 6.150

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