{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Xia A"],"funding":["NICHD NIH HHS","NIDCD NIH HHS","NINDS NIH HHS"],"pagination":["209-23"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC2869304"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["3(3-4)"],"pubmed_abstract":["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 mutation in mice and found that the TM was shortened in heterozygous Tecta(C1509G/+) mice, reaching only the first row of OHCs. Thus, deficient forward transduction renders OHCs within the second and third rows non-functional, producing partial hearing loss. Surprisingly, both Tecta(C1509G/+) and Tecta(C1509G/C1509G) mice were found to have increased reverse transduction as assessed b"],"journal":["Disease models & mechanisms"],"pubmed_title":["Deficient forward transduction and enhanced reverse transduction in the alpha tectorin C1509G human hearing loss mutation."],"pmcid":["PMC2869304"],"funding_grant_id":["F32 DC000354","DC008134","HD24064","R01 DC000354","DC00354","K08 DC006671","5K08NS53419","K08 NS053419","R01 DC009622","P30 HD024064","R21 DC008134"],"pubmed_authors":["Yuan T","Pereira FA","Pfister M","Xia A","Osborn A","Gao SS","Oghalai JS","Bress A","Maricich SM"],"additional_accession":[]},"is_claimable":false,"name":"Deficient forward transduction and enhanced reverse transduction in the alpha tectorin C1509G human hearing loss mutation.","description":"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 mutation in mice and found that the TM was shortened in heterozygous Tecta(C1509G/+) mice, reaching only the first row of OHCs. Thus, deficient forward transduction renders OHCs within the second and third rows non-functional, producing partial hearing loss. Surprisingly, both Tecta(C1509G/+) and Tecta(C1509G/C1509G) mice were found to have increased reverse transduction as assessed b","dates":{"release":"2010-01-01T00:00:00Z","publication":"2010 Mar-Apr","modification":"2026-07-15T14:27:55.062Z","creation":"2026-07-05T03:12:34.082Z"},"accession":"S-EPMC2869304","cross_references":{"pubmed":["20142329"],"doi":["10.1242/dmm.004135"]}}