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Article

Deficient forward transduction and enhanced reverse transduction in the alpha tectorin C1509G human hearing loss mutation.

Disease models & mechanisms - 1 Jan 2000

Xia Anping, Gao Simon S, Yuan Tao, Osborn Alexander, Bress Andreas, Pfister Markus, Maricich Stephen M, Pereira Fred A, Oghalai John S

Abstract excerpt

Most forms of hearing loss are associated with loss of cochlear outer hair cells (OHCs). OHCs require the tectorial membrane (TM) for stereociliary bundle stimulation (forward transduction) and active feedback (reverse transduction). Alpha tectorin is a protein constituent of the TM and the C1509G mutation in alpha tectorin in humans results in autosomal dominant hearing loss. We engineered and validated this...

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Identifiers

PMID: 20142329
DOI: 10.1242/dmm.004135
PMCID: PMC2869304

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