Dear Editor,Afferent synapses of cochlear inner hair cells(IHCs)employ a unique molecular machinery(see extended background in Supplementary Materials).Otoferlin is a key player in this machinery and its defects cause...Dear Editor,Afferent synapses of cochlear inner hair cells(IHCs)employ a unique molecular machinery(see extended background in Supplementary Materials).Otoferlin is a key player in this machinery and its defects cause human auditory synaptopathy(Moser and Starr,2016).Otoferlin,a tail-anchored(Vogl et al.,2016)multi-C_(2)-domain protein(Fig.1Ai)specific to hair cells(Roux et al.,2006),is a member of the ferlin protein family involved in membrane trafficking and repair that are of major disease relevance(Pangršičet al.,2012),also see Supplementary Materials.Otoferlin is distributed broadly within IHCs(Fig.2Ai-Aiii;Pangrsic et al.,2010;Roux et al.,2006).展开更多
基金supported by the Deutsche Forschungsgemeinschaft(DFG,German Research Foundation),under Germany’s Excellence Strategy—EXC 2067/1-390729940(to T.M.,N.B.and J.P.),via the Collaborative Research Center 889(to N.S.,C.W.,J.P.,N.B.and T.M.),via DFG VO 2138/7‐1(to B.V.),and via the Leibniz Program(to T.M.)supported by Fondation Pour l’Audition(FPA RD-2020-10)to T.M.Fundação de AmparoàPesquisa do Estado de São Paulo(CEPID 2013/08028-1)to R.C.M.N..
文摘Dear Editor,Afferent synapses of cochlear inner hair cells(IHCs)employ a unique molecular machinery(see extended background in Supplementary Materials).Otoferlin is a key player in this machinery and its defects cause human auditory synaptopathy(Moser and Starr,2016).Otoferlin,a tail-anchored(Vogl et al.,2016)multi-C_(2)-domain protein(Fig.1Ai)specific to hair cells(Roux et al.,2006),is a member of the ferlin protein family involved in membrane trafficking and repair that are of major disease relevance(Pangršičet al.,2012),also see Supplementary Materials.Otoferlin is distributed broadly within IHCs(Fig.2Ai-Aiii;Pangrsic et al.,2010;Roux et al.,2006).
