TMC1 and TMC2 are components of the mechanotransduction channel in hair cells of the mammalian inner ear

Neuron

Volumn 79, Issue 3, 2013, Pages 504-515

  Pan, Bifeng ^a   Géléoc, Gwenaelle S ^a   Asai, Yukako ^a   Horwitz, Geoffrey C ^a   Kurima, Kiyoto ^b   Ishikawa, Kotaro ^b   Kawashima, Yoshiyuki ^b   Griffith, Andrew J ^b   Holt, Jeffrey R ^a  

^a BOSTON CHILDREN S HOSPITAL   (United States)

^b NATIONAL INSTITUTE ON DEAFNESS AND OTHER COMMUNICATION DISORDERS   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CALCIUM; MEMBRANE PROTEIN; TMC1 PROTEIN; TMC2 PROTEIN; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ARTICLE; CALCIUM TRANSPORT; CONTROLLED STUDY; EVOKED BRAIN STEM AUDITORY RESPONSE; GENE; GENE EXPRESSION; HAIR CELL; INNER EAR; MECHANOTRANSDUCTION; MOUSE; NONHUMAN; POINT MUTATION; PRIORITY JOURNAL; STEREOCILIUM; TRANSMEMBRANE CHANNEL LIKE 1 GENE; TRANSMEMBRANE CHANNEL LIKE 2 GENE;

ACOUSTIC STIMULATION; ADENOVIRIDAE; AGE FACTORS; ANIMALS; AUDITORY PERCEPTION; BIOPHYSICAL PHENOMENA; CALCIUM; CELL COUNT; CELLS, CULTURED; DOSE-RESPONSE RELATIONSHIP, DRUG; EVOKED POTENTIALS, AUDITORY, BRAIN STEM; HAIR CELLS, AUDITORY; MECHANOTRANSDUCTION, CELLULAR; MEMBRANE POTENTIALS; MEMBRANE PROTEINS; MICE; MICE, TRANSGENIC; MUTATION; ORGAN OF CORTI; PATCH-CLAMP TECHNIQUES; TRANSDUCTION, GENETIC;

EID: 84881559173     PISSN: 08966273     EISSN: 10974199     Source Type: Journal    
DOI: 10.1016/j.neuron.2013.06.019     Document Type: Article

References (41)

1. Amalfitano A., Hauser M.A., Hu H., Serra D., Begy C.R., Chamberlain J.S. Production and characterization of improved adenovirus vectors with the E1, E2b, and E3 genes deleted. J.Virol. 1998, 72:926-933.
2. Arnadóttir J., Chalfie M. Eukaryotic mechanosensitive channels. Annu. Rev. Biophys. 2010, 39:111-137.
3. Assad J.A., Corey D.P. An active motor model for adaptation by vertebrate hair cells. J.Neurosci. 1992, 12:3291-3309.
4. Beurg M., Evans M.G., Hackney C.M., Fettiplace R. A large-conductance calcium-selective mechanotransducer channel in mammalian cochlear hair cells. J.Neurosci. 2006, 26:10992-11000.
5. Beurg M., Fettiplace R., Nam J.H., Ricci A.J. Localization of inner hair cell mechanotransducer channels using high-speed calcium imaging. Nat. Neurosci. 2009, 12:553-558.
6. Chatzigeorgiou M., Bang S., Hwang S.W., Schafer W.R. tmc-1 encodes a sodium-sensitive channel required for salt chemosensation in C. elegans. Nature 2013, 494:95-99.
7. Christensen A.P., Corey D.P. TRP channels in mechanosensation: direct or indirect activation?. Nat. Rev. Neurosci. 2007, 8:510-521.
8. Corey D.P., Hudspeth A.J. Kinetics of the receptor current in bullfrog saccular hair cells. J.Neurosci. 1983, 3:962-976.
9. Crawford A.C., Evans M.G., Fettiplace R. The actions of calcium on the mechano-electrical transducer current of turtle hair cells. J.Physiol. 1991, 434:369-398.
10. Denk W., Holt J.R., Shepherd G.M., Corey D.P. Calcium imaging of single stereocilia in hair cells: localization of transduction channels at both ends of tip links. Neuron 1995, 15:1311-1321.
11. Eatock R.A., Corey D.P., Hudspeth A.J. Adaptation of mechanoelectrical transduction in hair cells of the bullfrog's sacculus. J.Neurosci. 1987, 7:2821-2836.
12. Esterberg R., Hailey D.W., Coffin A.B., Raible D.W., Rubel E.W. Disruption of intracellular calcium regulation is integral to aminoglycoside-induced hair cell death. J.Neurosci. 2013, 33:7513-7525.
13. Farris H.E., Wells G.B., Ricci A.J. Steady-state adaptation of mechanotransduction modulates the resting potential of auditory hair cells, providing an assay for endolymph [Ca2+]. J.Neurosci. 2006, 26:12526-12536.
14. Géléoc G.S., Lennan G.W., Richardson G.P., Kros C.J. A quantitative comparison of mechanoelectrical transduction in vestibular and auditory hair cells of neonatal mice. Proc. Biol. Sci. 1997, 264:611-621.
15. Hodges B.L., Serra D., Hu H., Begy C.A., Chamberlain J.S., Amalfitano A. Multiply deleted [E1, polymerase-, and pTP-] adenovirus vector persists despite deletion of the preterminal protein. J.Gene Med. 2000, 2:250-259.
16. Holt J.R., Gillespie S.K., Provance D.W., Shah K., Shokat K.M., Corey D.P., Mercer J.A., Gillespie P.G. A chemical-genetic strategy implicates myosin-1c in adaptation by hair cells. Cell 2002, 108:371-381.
17. Holton T., Hudspeth A.J. The transduction channel of hair cells from the bull-frog characterized by noise analysis. J.Physiol. 1986, 375:195-227.
18. Jaramillo F., Hudspeth A.J. Localization of the hair cell's transduction channels at the hair bundle's top by iontophoretic application of a channel blocker. Neuron 1991, 7:409-420.
19. Kang L., Gao J., Schafer W.R., Xie Z., Xu X.Z. C.elegans TRP family protein TRP-4 is a pore-forming subunit of a native mechanotransduction channel. Neuron 2010, 67:381-391.
20. Kawashima Y., Géléoc G.S., Kurima K., Labay V., Lelli A., Asai Y., Makishima T., Wu D.K., Della Santina C.C., Holt J.R., Griffith A.J. Mechanotransduction in mouse inner ear hair cells requires transmembrane channel-like genes. J.Clin. Invest. 2011, 121:4796-4809.
21. Kennedy H.J., Evans M.G., Crawford A.C., Fettiplace R. Fast adaptation of mechanoelectrical transducer channels in mammalian cochlear hair cells. Nat. Neurosci. 2003, 6:832-836.
22. Kim K.X., Fettiplace R. Developmental changes in the cochlear hair cell mechanotransducer channel and their regulation by transmembrane channel-like proteins. J.Gen. Physiol. 2013, 141:141-148.
23. Kubisch C., Schroeder B.C., Friedrich T., Lütjohann B., El-Amraoui A., Marlin S., Petit C., Jentsch T.J. KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness. Cell 1999, 96:437-446.
24. Kurima K., Peters L.M., Yang Y., Riazuddin S., Ahmed Z.M., Naz S., Arnaud D., Drury S., Mo J., Makishima T., et al. Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function. Nat. Genet. 2002, 30:277-284.
25. Labay V., Weichert R.M., Makishima T., Griffith A.J. Topology of transmembrane channel-like gene 1 protein. Biochemistry 2010, 49:8592-8598.
26. Lelli A., Asai Y., Forge A., Holt J.R., Géléoc G.S. Tonotopic gradient in the developmental acquisition of sensory transduction in outer hair cells of the mouse cochlea. J.Neurophysiol. 2009, 101:2961-2973.
27. 2+ entry through mechanosensitive channels localizes the site of mechanoelectrical transduction in hair cells. Proc. Natl. Acad. Sci. USA 1995, 92:10297-10301.
28. Marcotti W., van Netten S.M., Kros C.J. The aminoglycoside antibiotic dihydrostreptomycin rapidly enters mouse outer hair cells through the mechano-electrical transducer channels. J.Physiol. 2005, 567:505-521.
29. Marcotti W., Erven A., Johnson S.L., Steel K.P., Kros C.J. Tmc1 is necessary for normal functional maturation and survival of inner and outer hair cells in the mouse cochlea. J.Physiol. 2006, 574:677-698.
30. O'Hagan R., Chalfie M., Goodman M.B. The MEC-4 DEG/ENaC channel of Caenorhabditis elegans touch receptor neurons transduces mechanical signals. Nat. Neurosci. 2005, 8:43-50.
31. Ricci A.J., Fettiplace R. The effects of calcium buffering and cyclic AMP on mechano-electrical transduction in turtle auditory hair cells. J.Physiol. 1997, 501:111-124.
32. Ricci A.J., Crawford A.C., Fettiplace R. Tonotopic variation in the conductance of the hair cell mechanotransducer channel. Neuron 2003, 40:983-990.
33. Rüsch A., Kros C.J., Richardson G.P. Block by amiloride and its derivatives of mechano-electrical transduction in outer hair cells of mouse cochlear cultures. J.Physiol. 1994, 474:75-86.
34. Stauffer E.A., Holt J.R. Sensory transduction and adaptation in inner and outer hair cells of the mouse auditory system. J.Neurophysiol. 2007, 98:3360-3369.
35. Steel K.P., Barkway C. Another role for melanocytes: their importance for normal stria vascularis development in the mammalian inner ear. Development 1989, 107:453-463.
36. Vreugde S., Erven A., Kros C.J., Marcotti W., Fuchs H., Kurima K., Wilcox E.R., Friedman T.B., Griffith A.J., Balling R., et al. Beethoven, a mouse model for dominant, progressive hearing loss DFNA36. Nat. Genet. 2002, 30:257-258.
37. Waguespack J., Salles F.T., Kachar B., Ricci A.J. Stepwise morphological and functional maturation of mechanotransduction in rat outer hair cells. J.Neurosci. 2007, 27:13890-13902.
38. Wu Y.C., Ricci A.J., Fettiplace R. Two components of transducer adaptation in auditory hair cells. J.Neurophysiol. 1999, 82:2171-2181.
39. Xiong W., Grillet N., Elledge H.M., Wagner T.F., Zhao B., Johnson K.R., Kazmierczak P., Müller U. TMHS is an integral component of the mechanotransduction machinery of cochlear hair cells. Cell 2012, 151:1283-1295.
40. Yan Z., Zhang W., He Y., Gorczyca D., Xiang Y., Cheng L.E., Meltzer S., Jan L.Y., Jan Y.N. Drosophila NOMPC is a mechanotransduction channel subunit for gentle-touch sensation. Nature 2013, 493:221-225.
41. Yang T., Kahrizi K., Bazazzadeghan N., Meyer N., Najmabadi H., Smith R.J. A novel mutation adjacent to the Bth mouse mutation in the TMC1 gene makes this mouse an excellent model of human deafness at the DFNA36 locus. Clin. Genet. 2010, 77:395-398.