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Literature DB >> 30291150

Function and Dysfunction of TMC Channels in Inner Ear Hair Cells.

David P Corey¹, Nurunisa Akyuz¹, Jeffrey R Holt².

Abstract

The TMC1 channel was identified as a protein essential for hearing in mouse and human, and recognized as one of a family of eight such proteins in mammals. The TMC family is part of a superfamily of seven branches, which includes the TMEM16s. Vertebrate hair cells express both TMC1 and TMC2. They are located at the tips of stereocilia and are required for hair cell mechanotransduction. TMC1 assembles as a dimer and its similarity to the TMEM16s has enabled a predicted tertiary structure with an ion conduction pore in each subunit of the dimer. Cysteine mutagenesis of the pore supports the role of TMC1 and TMC2 as the core channel proteins of a larger mechanotransduction complex that includes PCDH15 and LHFPL5, and perhaps TMIE, CIB2 and others.

Entities: CellLine Chemical Disease Gene Mutation Species

Year: 2019 PMID： 30291150 PMCID： PMC6450785 DOI： 10.1101/cshperspect.a033506

Source DB: PubMed Journal: Cold Spring Harb Perspect Med ISSN： 2157-1422 Impact factor: 6.915

80 in total

1. A large-conductance calcium-selective mechanotransducer channel in mammalian cochlear hair cells.

Authors: Maryline Beurg; Michael G Evans; Carole M Hackney; Robert Fettiplace
Journal: J Neurosci Date: 2006-10-25 Impact factor: 6.167

2. Kinetics of the receptor current in bullfrog saccular hair cells.

Authors: D P Corey; A J Hudspeth
Journal: J Neurosci Date: 1983-05 Impact factor: 6.167

3. Mechanotransduction by PCDH15 Relies on a Novel cis-Dimeric Architecture.

Authors: Gilman Dionne; Xufeng Qiu; Micah Rapp; Xiaoping Liang; Bo Zhao; Guihong Peng; Phinikoula S Katsamba; Goran Ahlsen; Rotem Rubinstein; Clinton S Potter; Bridget Carragher; Barry Honig; Ulrich Müller; Lawrence Shapiro
Journal: Neuron Date: 2018-07-26 Impact factor: 17.173

4. Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function.

Authors: Kiyoto Kurima; Linda M Peters; Yandan Yang; Saima Riazuddin; Zubair M Ahmed; Sadaf Naz; Deidre Arnaud; Stacy Drury; Jianhong Mo; Tomoko Makishima; Manju Ghosh; P S N Menon; Dilip Deshmukh; Carole Oddoux; Harry Ostrer; Shaheen Khan; Sheikh Riazuddin; Prescott L Deininger; Lori L Hampton; Susan L Sullivan; James F Battey; Bronya J B Keats; Edward R Wilcox; Thomas B Friedman; Andrew J Griffith
Journal: Nat Genet Date: 2002-02-19 Impact factor: 38.330

5. Mutation of the novel gene Tmie results in sensory cell defects in the inner ear of spinner, a mouse model of human hearing loss DFNB6.

Authors: Kristina L Mitchem; Ellen Hibbard; Lisa A Beyer; Ken Bosom; Gary A Dootz; David F Dolan; Kenneth R Johnson; Yehoash Raphael; David C Kohrman
Journal: Hum Mol Genet Date: 2002-08-01 Impact factor: 6.150

6. TMC1 and TMC2 Localize at the Site of Mechanotransduction in Mammalian Inner Ear Hair Cell Stereocilia.

Authors: Kiyoto Kurima; Seham Ebrahim; Bifeng Pan; Miloslav Sedlacek; Prabuddha Sengupta; Bryan A Millis; Runjia Cui; Hiroshi Nakanishi; Taro Fujikawa; Yoshiyuki Kawashima; Byung Yoon Choi; Kelly Monahan; Jeffrey R Holt; Andrew J Griffith; Bechara Kachar
Journal: Cell Rep Date: 2015-08-28 Impact factor: 9.423

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

Authors: Bifeng Pan; Gwenaelle S Géléoc; Yukako Asai; Geoffrey C Horwitz; Kiyoto Kurima; Kotaro Ishikawa; Yoshiyuki Kawashima; Andrew J Griffith; Jeffrey R Holt
Journal: Neuron Date: 2013-07-18 Impact factor: 17.173

8. The nature of inherited deafness in deafness mice.

Authors: K P Steel; G R Bock
Journal: Nature Date: 1980-11-13 Impact factor: 49.962

Review 9. Is TMC1 the Hair Cell Mechanotransducer Channel?

Authors: Robert Fettiplace
Journal: Biophys J Date: 2016-07-12 Impact factor: 4.033

10. Structure of mouse protocadherin 15 of the stereocilia tip link in complex with LHFPL5.

Authors: Jingpeng Ge; Johannes Elferich; April Goehring; Huaying Zhao; Peter Schuck; Eric Gouaux
Journal: Elife Date: 2018-08-02 Impact factor: 8.140

17 in total

1. TMC1 Forms the Pore of Mechanosensory Transduction Channels in Vertebrate Inner Ear Hair Cells.

Authors: Bifeng Pan; Nurunisa Akyuz; Xiao-Ping Liu; Yukako Asai; Carl Nist-Lund; Kiyoto Kurima; Bruce H Derfler; Bence György; Walrati Limapichat; Sanket Walujkar; Lahiru N Wimalasena; Marcos Sotomayor; David P Corey; Jeffrey R Holt
Journal: Neuron Date: 2018-08-22 Impact factor: 17.173

2. Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Authors: Sophie Boucher; Fabienne Wong Jun Tai; Sedigheh Delmaghani; Andrea Lelli; Amrit Singh-Estivalet; Typhaine Dupont; Magali Niasme-Grare; Vincent Michel; Nicolas Wolff; Amel Bahloul; Yosra Bouyacoub; Didier Bouccara; Bernard Fraysse; Olivier Deguine; Lionel Collet; Hung Thai-Van; Eugen Ionescu; Jean-Louis Kemeny; Fabrice Giraudet; Jean-Pierre Lavieille; Arnaud Devèze; Anne-Laure Roudevitch-Pujol; Christophe Vincent; Christian Renard; Valérie Franco-Vidal; Claire Thibult-Apt; Vincent Darrouzet; Eric Bizaguet; Arnaud Coez; Hugues Aschard; Nicolas Michalski; Gaëlle M Lefevre; Anne Aubois; Paul Avan; Crystel Bonnet; Christine Petit
Journal: Proc Natl Acad Sci U S A Date: 2020-11-23 Impact factor: 11.205

Function and Dysfunction of TMC Channels in Inner Ear Hair Cells.

1. A large-conductance calcium-selective mechanotransducer channel in mammalian cochlear hair cells.

2. Kinetics of the receptor current in bullfrog saccular hair cells.

3. Mechanotransduction by PCDH15 Relies on a Novel cis-Dimeric Architecture.

4. Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function.

5. Mutation of the novel gene Tmie results in sensory cell defects in the inner ear of spinner, a mouse model of human hearing loss DFNB6.

6. TMC1 and TMC2 Localize at the Site of Mechanotransduction in Mammalian Inner Ear Hair Cell Stereocilia.

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

8. The nature of inherited deafness in deafness mice.

Review 9. Is TMC1 the Hair Cell Mechanotransducer Channel?

10. Structure of mouse protocadherin 15 of the stereocilia tip link in complex with LHFPL5.

1. TMC1 Forms the Pore of Mechanosensory Transduction Channels in Vertebrate Inner Ear Hair Cells.

2. Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Review 3. Aminoglycoside- and Cisplatin-Induced Ototoxicity: Mechanisms and Otoprotective Strategies.

Review 4. Distinct functions of TMC channels: a comparative overview.

5. Mechanotransduction Ion Channels in Hearing and Touch.

6. Mechanosensitive Ion Channels, Axonal Growth, and Regeneration.

Review 7. Inner Ear Connexin Channels: Roles in Development and Maintenance of Cochlear Function.

Review 8. How Transmembrane Inner Ear (TMIE) plays role in the auditory system: A mystery to us.

9. Improved TMC1 gene therapy restores hearing and balance in mice with genetic inner ear disorders.

Review 10. Practical aspects of inner ear gene delivery for research and clinical applications.