Mice deficient in H+-ATPase a4 subunit have severe hearing impairment associated with enlarged endolymphatic compartments within the inner ear

Mutations in the ATP6V0A4 gene lead to autosomal recessive distal renal tubular acidosis in patients, who often show sensorineural hearing impairment. A first Atp6v0a4 knockout mouse model that recapitulates the loss of H+-ATPase function seen in humans has been generated and recently reported (Norg...

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Bibliographic Details
Main Authors: Lorente-Cánovas, Beatriz, Ingham, Neil, Norgett, Elizabeth E., Golder, Zoe J., Karet Frankl, Fiona E., Steel, Karen P.
Format: Online
Language:English
Published: The Company of Biologists Limited 2013
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3597025/