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10.1016/j.anl.2014.04.001 http://scihub22266oqcxt.onion/10.1016/j.anl.2014.04.001 C4176506!4176506!24933710     free     free     free Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Auris+Nasus+Larynx 2014 ; 41 (5): 399-408 Nephropedia Template TP {{tp|p=24933710|t=2014. Mutations of TMC1 cause deafness by disrupting mechanoelectrical transduction |pdf=|usr=}}{{24933710}} gab.com Text Mutations of TMC1 cause deafness by disrupting mechanoelectrical transduction JO: Auris Nasus Larynx http://www.kidney.de/pget.php?&tw=1&pmid=24933710 #FOAvip Objective: Mutations of transmembrane channel-like 1 gene (TMC1) can cause dominant (DFNA36) or recessive (DFNB7/B11) deafness. In this article, we describe the characteristics of DFNA36 and DFNB7/B11 deafness, the features of the Tmc1 mutant mouse strains, and recent advances in our understanding of TMC1 function. Methods: Publications related to TMC1, DFNA36 or DFNB7/B11 were identified through PubMed. Results: All affected DFNA36 subjects showed post-lingual, progressive, sensorineural hearing loss (HL), initially affecting high frequencies. In contrast, almost all affected DFNB7/B11 subjects demonstrated congenital or prelingual severe to profound sensorineural HL. The mouse Tmc1 gene also has dominant and recessive mutant alleles that cause HL in mutant strains, including Beethoven, deafness and Tmc1 knockout mice. These mutant mice have been instrumental for revealing that Tmc1 and its closely related paralog Tmc2 are expressed in cochlear and vestibular hair cells, and are required for hair cell mechanoelectrical transduction (MET). Recent studies suggest that TMC1 and TMC2 may be components of the long-sought hair cell MET channel. Conclusion: TMC1 mutations disrupt hair cell MET. Twit Text FOAVip Mutations of TMC1 cause deafness by disrupting mechanoelectrical transduction ????Auris Nasus Larynx http://www.kidney.de/pget.php?&tw=1&pmid=24933710 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=24933710 #FOAvip English Wikipedia <ref>{{Cite pmid|24933710}}</ref> Mutations of TMC1 cause deafness by disrupting mechanoelectrical transduction #MMPMID24933710 Nakanishi H; Kurima K; Kawashima Y; Griffith AJ Auris Nasus Larynx 2014[Oct]; 41 (5): 399-408 PMID24933710show ga Objective: Mutations of transmembrane channel-like 1 gene (TMC1) can cause dominant (DFNA36) or recessive (DFNB7/B11) deafness. In this article, we describe the characteristics of DFNA36 and DFNB7/B11 deafness, the features of the Tmc1 mutant mouse strains, and recent advances in our understanding of TMC1 function. Methods: Publications related to TMC1, DFNA36 or DFNB7/B11 were identified through PubMed. Results: All affected DFNA36 subjects showed post-lingual, progressive, sensorineural hearing loss (HL), initially affecting high frequencies. In contrast, almost all affected DFNB7/B11 subjects demonstrated congenital or prelingual severe to profound sensorineural HL. The mouse Tmc1 gene also has dominant and recessive mutant alleles that cause HL in mutant strains, including Beethoven, deafness and Tmc1 knockout mice. These mutant mice have been instrumental for revealing that Tmc1 and its closely related paralog Tmc2 are expressed in cochlear and vestibular hair cells, and are required for hair cell mechanoelectrical transduction (MET). Recent studies suggest that TMC1 and TMC2 may be components of the long-sought hair cell MET channel. Conclusion: TMC1 mutations disrupt hair cell MET. äMutations of TMC1 cause deafness by disrupting mechanoelectrical trans(epmc).pdf DeepDyve Pubget Overpricing