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Ultrastructural defects in stereocilia and tectorial membrane in aging mouse and human cochleae.

Bullen A, Forge A, Wright A, Richardson GP, Goodyear RJ, Taylor R.

J Neurosci Res. 2019 Nov 24. doi: 10.1002/jnr.24556. [Epub ahead of print]


Gentamicin Affects the Bioenergetics of Isolated Mitochondria and Collapses the Mitochondrial Membrane Potential in Cochlear Sensory Hair Cells.

O'Reilly M, Young L, Kirkwood NK, Richardson GP, Kros CJ, Moore AL.

Front Cell Neurosci. 2019 Sep 13;13:416. doi: 10.3389/fncel.2019.00416. eCollection 2019.


ORC-13661 protects sensory hair cells from aminoglycoside and cisplatin ototoxicity.

Kitcher SR, Kirkwood NK, Camci ED, Wu P, Gibson RM, Redila VA, Simon JA, Rubel EW, Raible DW, Richardson GP, Kros CJ.

JCI Insight. 2019 Aug 8;4(15). pii: 126764. doi: 10.1172/jci.insight.126764. eCollection 2019 Aug 8.


Accelerated Age-Related Degradation of the Tectorial Membrane in the Ceacam16βgal/βgal Null Mutant Mouse, a Model for Late-Onset Human Hereditary Deafness DFNB113.

Goodyear RJ, Cheatham MA, Naskar S, Zhou Y, Osgood RT, Zheng J, Richardson GP.

Front Mol Neurosci. 2019 Jun 12;12:147. doi: 10.3389/fnmol.2019.00147. eCollection 2019.


Design, Synthesis, and Biological Evaluation of a New Series of Carvedilol Derivatives That Protect Sensory Hair Cells from Aminoglycoside-Induced Damage by Blocking the Mechanoelectrical Transducer Channel.

O'Reilly M, Kirkwood NK, Kenyon EJ, Huckvale R, Cantillon DM, Waddell SJ, Ward SE, Richardson GP, Kros CJ, Derudas M.

J Med Chem. 2019 Jun 13;62(11):5312-5329. doi: 10.1021/acs.jmedchem.8b01325. Epub 2019 May 24.


Spontaneous Otoacoustic Emissions in TectaY1870C/+ Mice Reflect Changes in Cochlear Amplification and How It Is Controlled by the Tectorial Membrane.

Cheatham MA, Zhou Y, Goodyear RJ, Dallos P, Richardson GP.

eNeuro. 2018 Dec 26;5(6). pii: ENEURO.0314-18.2018. doi: 10.1523/ENEURO.0314-18.2018. eCollection 2018 Nov-Dec.


Hair-Bundle Links: Genetics as the Gateway to Function.

Richardson GP, Petit C.

Cold Spring Harb Perspect Med. 2019 Dec 2;9(12). pii: a033142. doi: 10.1101/cshperspect.a033142. Review.


FGFR1-mediated protocadherin-15 loading mediates cargo specificity during intraflagellar transport in inner ear hair-cell kinocilia.

Honda A, Kita T, Seshadri SV, Misaki K, Ahmed Z, Ladbury JE, Richardson GP, Yonemura S, Ladher RK.

Proc Natl Acad Sci U S A. 2018 Aug 14;115(33):8388-8393. doi: 10.1073/pnas.1719861115. Epub 2018 Jul 30.


Structure, Function, and Development of the Tectorial Membrane: An Extracellular Matrix Essential for Hearing.

Goodyear RJ, Richardson GP.

Curr Top Dev Biol. 2018;130:217-244. doi: 10.1016/bs.ctdb.2018.02.006. Epub 2018 Mar 26. Review.


Identification of ion-channel modulators that protect against aminoglycoside-induced hair cell death.

Kenyon EJ, Kirkwood NK, Kitcher SR, O'Reilly M, Derudas M, Cantillon DM, Goodyear RJ, Secker A, Baxendale S, Bull JC, Waddell SJ, Whitfield TT, Ward SE, Kros CJ, Richardson GP.

JCI Insight. 2017 Dec 21;2(24). pii: 96773. doi: 10.1172/jci.insight.96773.


TMC2 Modifies Permeation Properties of the Mechanoelectrical Transducer Channel in Early Postnatal Mouse Cochlear Outer Hair Cells.

Corns LF, Jeng JY, Richardson GP, Kros CJ, Marcotti W.

Front Mol Neurosci. 2017 Oct 18;10:326. doi: 10.3389/fnmol.2017.00326. eCollection 2017.


The temporal expression profile of a Nos3-related natural antisense RNA in the brain suggests a possible role in neurogenesis.

Chavez GG, Taylor G, Garaliene J, Richardson GP, Korneev SA.

Nitric Oxide. 2017 Dec 1;71:27-31. doi: 10.1016/j.niox.2017.10.002. Epub 2017 Oct 12.


A tectorin-based matrix and planar cell polarity genes are required for normal collagen-fibril orientation in the developing tectorial membrane.

Goodyear RJ, Lu X, Deans MR, Richardson GP.

Development. 2017 Nov 1;144(21):3978-3989. doi: 10.1242/dev.151696. Epub 2017 Sep 21.


d-Tubocurarine and Berbamine: Alkaloids That Are Permeant Blockers of the Hair Cell's Mechano-Electrical Transducer Channel and Protect from Aminoglycoside Toxicity.

Kirkwood NK, O'Reilly M, Derudas M, Kenyon EJ, Huckvale R, van Netten SM, Ward SE, Richardson GP, Kros CJ.

Front Cell Neurosci. 2017 Sep 5;11:262. doi: 10.3389/fncel.2017.00262. eCollection 2017.


Easi-CRISPR: a robust method for one-step generation of mice carrying conditional and insertion alleles using long ssDNA donors and CRISPR ribonucleoproteins.

Quadros RM, Miura H, Harms DW, Akatsuka H, Sato T, Aida T, Redder R, Richardson GP, Inagaki Y, Sakai D, Buckley SM, Seshacharyulu P, Batra SK, Behlke MA, Zeiner SA, Jacobi AM, Izu Y, Thoreson WB, Urness LD, Mansour SL, Ohtsuka M, Gurumurthy CB.

Genome Biol. 2017 May 17;18(1):92. doi: 10.1186/s13059-017-1220-4.


Protecting Mammalian Hair Cells from Aminoglycoside-Toxicity: Assessing Phenoxybenzamine's Potential.

Majumder P, Moore PA, Richardson GP, Gale JE.

Front Cell Neurosci. 2017 Apr 18;11:94. doi: 10.3389/fncel.2017.00094. eCollection 2017.


Cell-Cell Contact Area Affects Notch Signaling and Notch-Dependent Patterning.

Shaya O, Binshtok U, Hersch M, Rivkin D, Weinreb S, Amir-Zilberstein L, Khamaisi B, Oppenheim O, Desai RA, Goodyear RJ, Richardson GP, Chen CS, Sprinzak D.

Dev Cell. 2017 Mar 13;40(5):505-511.e6. doi: 10.1016/j.devcel.2017.02.009.


The acquisition of mechano-electrical transducer current adaptation in auditory hair cells requires myosin VI.

Marcotti W, Corns LF, Goodyear RJ, Rzadzinska AK, Avraham KB, Steel KP, Richardson GP, Kros CJ.

J Physiol. 2016 Jul 1;594(13):3667-81. doi: 10.1113/JP272220. Epub 2016 May 27.


Increased Spontaneous Otoacoustic Emissions in Mice with a Detached Tectorial Membrane.

Cheatham MA, Ahmad A, Zhou Y, Goodyear RJ, Dallos P, Richardson GP.

J Assoc Res Otolaryngol. 2016 Apr;17(2):81-8. doi: 10.1007/s10162-015-0551-7. Epub 2015 Dec 21.


A novel long non-coding natural antisense RNA is a negative regulator of Nos1 gene expression.

Korneev SA, Maconochie M, Naskar S, Korneeva EI, Richardson GP, O'Shea M.

Sci Rep. 2015 Jul 8;5:11815. doi: 10.1038/srep11815.


Loss of the tectorial membrane protein CEACAM16 enhances spontaneous, stimulus-frequency, and transiently evoked otoacoustic emissions.

Cheatham MA, Goodyear RJ, Homma K, Legan PK, Korchagina J, Naskar S, Siegel JH, Dallos P, Zheng J, Richardson GP.

J Neurosci. 2014 Jul 30;34(31):10325-38. doi: 10.1523/JNEUROSCI.1256-14.2014.


Transduction without tip links in cochlear hair cells is mediated by ion channels with permeation properties distinct from those of the mechano-electrical transducer channel.

Marcotti W, Corns LF, Desmonds T, Kirkwood NK, Richardson GP, Kros CJ.

J Neurosci. 2014 Apr 16;34(16):5505-14. doi: 10.1523/JNEUROSCI.4086-13.2014.


Staurosporine-induced collapse of cochlear hair bundles.

Goodyear RJ, Ratnayaka HS, Warchol ME, Richardson GP.

J Comp Neurol. 2014 Oct 1;522(14):3281-94. doi: 10.1002/cne.23597. Epub 2014 Apr 17.


Porosity controls spread of excitation in tectorial membrane traveling waves.

Sellon JB, Ghaffari R, Farrahi S, Richardson GP, Freeman DM.

Biophys J. 2014 Mar 18;106(6):1406-13. doi: 10.1016/j.bpj.2014.02.012.


Three deaf mice: mouse models for TECTA-based human hereditary deafness reveal domain-specific structural phenotypes in the tectorial membrane.

Legan PK, Goodyear RJ, Morín M, Mencia A, Pollard H, Olavarrieta L, Korchagina J, Modamio-Hoybjor S, Mayo F, Moreno F, Moreno-Pelayo MA, Richardson GP.

Hum Mol Genet. 2014 May 15;23(10):2551-68. doi: 10.1093/hmg/ddt646. Epub 2013 Dec 20.


Progressive hearing loss and gradual deterioration of sensory hair bundles in the ears of mice lacking the actin-binding protein Eps8L2.

Furness DN, Johnson SL, Manor U, Rüttiger L, Tocchetti A, Offenhauser N, Olt J, Goodyear RJ, Vijayakumar S, Dai Y, Hackney CM, Franz C, Di Fiore PP, Masetto S, Jones SM, Knipper M, Holley MC, Richardson GP, Kachar B, Marcotti W.

Proc Natl Acad Sci U S A. 2013 Aug 20;110(34):13898-903. doi: 10.1073/pnas.1304644110. Epub 2013 Aug 5.


A mouse model for human deafness DFNB22 reveals that hearing impairment is due to a loss of inner hair cell stimulation.

Lukashkin AN, Legan PK, Weddell TD, Lukashkina VA, Goodyear RJ, Welstead LJ, Petit C, Russell IJ, Richardson GP.

Proc Natl Acad Sci U S A. 2012 Nov 20;109(47):19351-6. doi: 10.1073/pnas.1210159109. Epub 2012 Nov 5.


A mutation in PNPT1, encoding mitochondrial-RNA-import protein PNPase, causes hereditary hearing loss.

von Ameln S, Wang G, Boulouiz R, Rutherford MA, Smith GM, Li Y, Pogoda HM, Nürnberg G, Stiller B, Volk AE, Borck G, Hong JS, Goodyear RJ, Abidi O, Nürnberg P, Hofmann K, Richardson GP, Hammerschmidt M, Moser T, Wollnik B, Koehler CM, Teitell MA, Barakat A, Kubisch C.

Am J Hum Genet. 2012 Nov 2;91(5):919-27. doi: 10.1016/j.ajhg.2012.09.002. Epub 2012 Oct 18.


The Brain Prize 2012: opening the doors of auditory perception.

Richardson GP.

Trends Neurosci. 2012 Sep;35(9):524-6. doi: 10.1016/j.tins.2012.06.005. Epub 2012 Jul 12.


TRPC3 and TRPC6 are essential for normal mechanotransduction in subsets of sensory neurons and cochlear hair cells.

Quick K, Zhao J, Eijkelkamp N, Linley JE, Rugiero F, Cox JJ, Raouf R, Gringhuis M, Sexton JE, Abramowitz J, Taylor R, Forge A, Ashmore J, Kirkwood N, Kros CJ, Richardson GP, Freichel M, Flockerzi V, Birnbaumer L, Wood JN.

Open Biol. 2012 May;2(5):120068. doi: 10.1098/rsob.120068.


Hair bundle defects and loss of function in the vestibular end organs of mice lacking the receptor-like inositol lipid phosphatase PTPRQ.

Goodyear RJ, Jones SM, Sharifi L, Forge A, Richardson GP.

J Neurosci. 2012 Feb 22;32(8):2762-72. doi: 10.1523/JNEUROSCI.3635-11.2012.


Mutations in protocadherin 15 and cadherin 23 affect tip links and mechanotransduction in mammalian sensory hair cells.

Alagramam KN, Goodyear RJ, Geng R, Furness DN, van Aken AF, Marcotti W, Kros CJ, Richardson GP.

PLoS One. 2011 Apr 21;6(4):e19183. doi: 10.1371/journal.pone.0019183.


Usher type 1G protein sans is a critical component of the tip-link complex, a structure controlling actin polymerization in stereocilia.

Caberlotto E, Michel V, Foucher I, Bahloul A, Goodyear RJ, Pepermans E, Michalski N, Perfettini I, Alegria-Prévot O, Chardenoux S, Do Cruzeiro M, Hardelin JP, Richardson GP, Avan P, Weil D, Petit C.

Proc Natl Acad Sci U S A. 2011 Apr 5;108(14):5825-30. doi: 10.1073/pnas.1017114108. Epub 2011 Mar 21.


Carcinoembryonic antigen-related cell adhesion molecule 16 interacts with alpha-tectorin and is mutated in autosomal dominant hearing loss (DFNA4).

Zheng J, Miller KK, Yang T, Hildebrand MS, Shearer AE, DeLuca AP, Scheetz TE, Drummond J, Scherer SE, Legan PK, Goodyear RJ, Richardson GP, Cheatham MA, Smith RJ, Dallos P.

Proc Natl Acad Sci U S A. 2011 Mar 8;108(10):4218-23. doi: 10.1073/pnas.1005842108. Epub 2011 Feb 22.


Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane.

Verpy E, Leibovici M, Michalski N, Goodyear RJ, Houdon C, Weil D, Richardson GP, Petit C.

J Comp Neurol. 2011 Feb 1;519(2):194-210. doi: 10.1002/cne.22509.


Tectorial membrane material properties in Tecta(Y)(1870C/+) heterozygous mice.

Masaki K, Ghaffari R, Gu JW, Richardson GP, Freeman DM, Aranyosi AJ.

Biophys J. 2010 Nov 17;99(10):3274-81. doi: 10.1016/j.bpj.2010.09.033.


How the genetics of deafness illuminates auditory physiology.

Richardson GP, de Monvel JB, Petit C.

Annu Rev Physiol. 2011;73:311-34. doi: 10.1146/annurev-physiol-012110-142228. Review.


Tectorial membrane travelling waves underlie abnormal hearing in Tectb mutant mice.

Ghaffari R, Aranyosi AJ, Richardson GP, Freeman DM.

Nat Commun. 2010 Oct 19;1:96. doi: 10.1038/ncomms1094.


Asymmetric distribution of cadherin 23 and protocadherin 15 in the kinocilial links of avian sensory hair cells.

Goodyear RJ, Forge A, Legan PK, Richardson GP.

J Comp Neurol. 2010 Nov 1;518(21):4288-97. doi: 10.1002/cne.22456.


Identification of the hair cell soma-1 antigen, HCS-1, as otoferlin.

Goodyear RJ, Legan PK, Christiansen JR, Xia B, Korchagina J, Gale JE, Warchol ME, Corwin JT, Richardson GP.

J Assoc Res Otolaryngol. 2010 Dec;11(4):573-86. doi: 10.1007/s10162-010-0231-6. Epub 2010 Aug 31.


Evidence for multiple, developmentally regulated isoforms of Ptprq on hair cells of the inner ear.

Nayak G, Goodyear RJ, Legan PK, Noda M, Richardson GP.

Dev Neurobiol. 2011 Feb;71(2):129-41. doi: 10.1002/dneu.20831.


Actin-bundling protein TRIOBP forms resilient rootlets of hair cell stereocilia essential for hearing.

Kitajiri S, Sakamoto T, Belyantseva IA, Goodyear RJ, Stepanyan R, Fujiwara I, Bird JE, Riazuddin S, Riazuddin S, Ahmed ZM, Hinshaw JE, Sellers J, Bartles JR, Hammer JA 3rd, Richardson GP, Griffith AJ, Frolenkov GI, Friedman TB.

Cell. 2010 May 28;141(5):786-98. doi: 10.1016/j.cell.2010.03.049.


Multiple roles for the tectorial membrane in the active cochlea.

Lukashkin AN, Richardson GP, Russell IJ.

Hear Res. 2010 Jul;266(1-2):26-35. doi: 10.1016/j.heares.2009.10.005. Epub 2009 Oct 21. Review.


Harmonin-b, an actin-binding scaffold protein, is involved in the adaptation of mechanoelectrical transduction by sensory hair cells.

Michalski N, Michel V, Caberlotto E, Lefèvre GM, van Aken AF, Tinevez JY, Bizard E, Houbron C, Weil D, Hardelin JP, Richardson GP, Kros CJ, Martin P, Petit C.

Pflugers Arch. 2009 Nov;459(1):115-30. doi: 10.1007/s00424-009-0711-x.


Linking genes underlying deafness to hair-bundle development and function.

Petit C, Richardson GP.

Nat Neurosci. 2009 Jun;12(6):703-10. doi: 10.1038/nn.2330. Epub 2009 May 26. Review.


Expression of the Pax2 transcription factor is associated with vestibular phenotype in the avian inner ear.

Warchol ME, Richardson GP.

Dev Neurobiol. 2009 Feb 1-15;69(2-3):191-202. doi: 10.1002/dneu.20684.


Stereocilin-deficient mice reveal the origin of cochlear waveform distortions.

Verpy E, Weil D, Leibovici M, Goodyear RJ, Hamard G, Houdon C, Lefèvre GM, Hardelin JP, Richardson GP, Avan P, Petit C.

Nature. 2008 Nov 13;456(7219):255-8. doi: 10.1038/nature07380. Epub 2008 Oct 8.


Aminoglycoside-induced phosphatidylserine externalization in sensory hair cells is regionally restricted, rapid, and reversible.

Goodyear RJ, Gale JE, Ranatunga KM, Kros CJ, Richardson GP.

J Neurosci. 2008 Oct 1;28(40):9939-52. doi: 10.1523/JNEUROSCI.1124-08.2008.


TRPML3 mutations cause impaired mechano-electrical transduction and depolarization by an inward-rectifier cation current in auditory hair cells of varitint-waddler mice.

van Aken AF, Atiba-Davies M, Marcotti W, Goodyear RJ, Bryant JE, Richardson GP, Noben-Trauth K, Kros CJ.

J Physiol. 2008 Nov 15;586(22):5403-18. doi: 10.1113/jphysiol.2008.156992. Epub 2008 Sep 18.


The tectorial membrane: one slice of a complex cochlear sandwich.

Richardson GP, Lukashkin AN, Russell IJ.

Curr Opin Otolaryngol Head Neck Surg. 2008 Oct;16(5):458-64. doi: 10.1097/MOO.0b013e32830e20c4. Review.

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