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Items: 42


Inhibition of Mitochondrial Division Attenuates Cisplatin-Induced Toxicity in the Neuromast Hair Cells.

Vargo JW, Walker SN, Gopal SR, Deshmukh AR, McDermott BM Jr, Alagramam KN, Stepanyan R.

Front Cell Neurosci. 2017 Dec 12;11:393. doi: 10.3389/fncel.2017.00393. eCollection 2017.


Spatiotemporal changes in the distribution of LHFPL5 in mice cochlear hair bundles during development and in the absence of PCDH15.

Mahendrasingam S, Fettiplace R, Alagramam KN, Cross E, Furness DN.

PLoS One. 2017 Oct 25;12(10):e0185285. doi: 10.1371/journal.pone.0185285. eCollection 2017.


Modeling and Preventing Progressive Hearing Loss in Usher Syndrome III.

Geng R, Omar A, Gopal SR, Chen DH, Stepanyan R, Basch ML, Dinculescu A, Furness DN, Saperstein D, Hauswirth W, Lustig LR, Alagramam KN.

Sci Rep. 2017 Oct 18;7(1):13480. doi: 10.1038/s41598-017-13620-9.


A small molecule mitigates hearing loss in a mouse model of Usher syndrome III.

Alagramam KN, Gopal SR, Geng R, Chen DH, Nemet I, Lee R, Tian G, Miyagi M, Malagu KF, Lock CJ, Esmieu WR, Owens AP, Lindsay NA, Ouwehand K, Albertus F, Fischer DF, B├╝rli RW, MacLeod AM, Harte WE, Palczewski K, Imanishi Y.

Nat Chem Biol. 2016 Jun;12(6):444-51. doi: 10.1038/nchembio.2069. Epub 2016 Apr 25.


Zebrafish Models for the Mechanosensory Hair Cell Dysfunction in Usher Syndrome 3 Reveal That Clarin-1 Is an Essential Hair Bundle Protein.

Gopal SR, Chen DH, Chou SW, Zang J, Neuhauss SC, Stepanyan R, McDermott BM Jr, Alagramam KN.

J Neurosci. 2015 Jul 15;35(28):10188-201. doi: 10.1523/JNEUROSCI.1096-15.2015.


Noise exposure immediately activates cochlear mitogen-activated protein kinase signaling.

Alagramam KN, Stepanyan R, Jamesdaniel S, Chen DH, Davis RR.

Noise Health. 2014 Nov-Dec;16(73):400-9. doi: 10.4103/1463-1741.144418.


A mouse model validates the utility of electrocochleography in verifying endolymphatic hydrops.

Melki SJ, Li Y, Semaan MT, Zheng QY, Megerian CA, Alagramam KN.

J Assoc Res Otolaryngol. 2014 Jun;15(3):413-21. doi: 10.1007/s10162-014-0445-0. Epub 2014 Feb 8.


ACF7 is a hair-bundle antecedent, positioned to integrate cuticular plate actin and somatic tubulin.

Antonellis PJ, Pollock LM, Chou SW, Hassan A, Geng R, Chen X, Fuchs E, Alagramam KN, Auer M, McDermott BM Jr.

J Neurosci. 2014 Jan 1;34(1):305-12. doi: 10.1523/JNEUROSCI.1880-13.2014.


Transient receptor potential melastatin 1: a hair cell transduction channel candidate.

Gerka-Stuyt J, Au A, Peachey NS, Alagramam KN.

PLoS One. 2013 Oct 11;8(10):e77213. doi: 10.1371/journal.pone.0077213. eCollection 2013.


Noddy, a mouse harboring a missense mutation in protocadherin-15, reveals the impact of disrupting a critical interaction site between tip-link cadherins in inner ear hair cells.

Geng R, Sotomayor M, Kinder KJ, Gopal SR, Gerka-Stuyt J, Chen DH, Hardisty-Hughes RE, Ball G, Parker A, Gaudet R, Furness D, Brown SD, Corey DP, Alagramam KN.

J Neurosci. 2013 Mar 6;33(10):4395-404. doi: 10.1523/JNEUROSCI.4514-12.2013.


The mechanosensory structure of the hair cell requires clarin-1, a protein encoded by Usher syndrome III causative gene.

Geng R, Melki S, Chen DH, Tian G, Furness DN, Oshima-Takago T, Neef J, Moser T, Askew C, Horwitz G, Holt JR, Imanishi Y, Alagramam KN.

J Neurosci. 2012 Jul 11;32(28):9485-98. doi: 10.1523/JNEUROSCI.0311-12.2012.


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.


Pharmacological protection of hearing loss in the mouse model of endolymphatic hydrops.

Melki SJ, Heddon CM, Frankel JK, Levitt AH, Momin SR, Alagramam KN, Megerian CA.

Laryngoscope. 2010 Aug;120(8):1637-45. doi: 10.1002/lary.21018.


Photodynamic therapy of cottontail rabbit papillomavirus-induced papillomas in a severe combined immunodeficient mouse xenograft system.

Lee RG, Vecchiotti MA, Heaphy J, Panneerselvam A, Schluchter MD, Oleinick NL, Lavertu P, Alagramam KN, Arnold JE, Sprecher RC.

Laryngoscope. 2010 Mar;120(3):618-24. doi: 10.1002/lary.20709.


Spiral ganglion loss outpaces inner hair cell loss in endolymphatic hydrops.

Momin SR, Melki SJ, Alagramam KN, Megerian CA.

Laryngoscope. 2010 Jan;120(1):159-65. doi: 10.1002/lary.20673.


A nonsynonymous SNP within PCDH15 is associated with lipid traits in familial combined hyperlipidemia.

Huertas-Vazquez A, Plaisier CL, Geng R, Haas BE, Lee J, Greevenbroek MM, van der Kallen C, de Bruin TW, Taskinen MR, Alagramam KN, Pajukanta P.

Hum Genet. 2010 Jan;127(1):83-9. doi: 10.1007/s00439-009-0749-z. Epub 2009 Oct 9.


Foreword for special issue: mouse models for hearing research.

Alagramam KN, Brown SD, Davis RR, Johnson KR, Jones SM, Macauley JB, Zheng QY, Zuo J.

Brain Res. 2009 Jun 24;1277:1-2. doi: 10.1016/j.brainres.2009.05.048. No abstract available.


Clarin-1, encoded by the Usher Syndrome III causative gene, forms a membranous microdomain: possible role of clarin-1 in organizing the actin cytoskeleton.

Tian G, Zhou Y, Hajkova D, Miyagi M, Dinculescu A, Hauswirth WW, Palczewski K, Geng R, Alagramam KN, Isosomppi J, Sankila EM, Flannery JG, Imanishi Y.

J Biol Chem. 2009 Jul 10;284(28):18980-93. doi: 10.1074/jbc.M109.003160. Epub 2009 May 7.


Usher syndrome IIIA gene clarin-1 is essential for hair cell function and associated neural activation.

Geng R, Geller SF, Hayashi T, Ray CA, Reh TA, Bermingham-McDonogh O, Jones SM, Wright CG, Melki S, Imanishi Y, Palczewski K, Alagramam KN, Flannery JG.

Hum Mol Genet. 2009 Aug 1;18(15):2748-60. doi: 10.1093/hmg/ddp210. Epub 2009 May 3.


Spiral ganglion degeneration patterns in endolymphatic hydrops.

Bixenstine PJ, Maniglia MP, Vasanji A, Alagramam KN, Megerian CA.

Laryngoscope. 2008 Jul;118(7):1217-23. doi: 10.1097/MLG.0b013e31816ba9cd.


A mouse model with postnatal endolymphatic hydrops and hearing loss.

Megerian CA, Semaan MT, Aftab S, Kisley LB, Zheng QY, Pawlowski KS, Wright CG, Alagramam KN.

Hear Res. 2008 Mar;237(1-2):90-105. doi: 10.1016/j.heares.2008.01.002. Epub 2008 Jan 15.


Promoter, alternative splice forms, and genomic structure of protocadherin 15.

Alagramam KN, Miller ND, Adappa ND, Pitts DR, Heaphy JC, Yuan H, Smith RJ.

Genomics. 2007 Oct;90(4):482-92. Epub 2007 Aug 15.


Tympanometry assessment of 61 inbred strains of mice.

Zheng QY, Tong YC, Alagramam KN, Yu H.

Hear Res. 2007 Sep;231(1-2):23-31. Epub 2007 Jun 2.


Molecular changes associated with the endolymphatic hydrops model.

Anne S, Kisley LB, Tajuddin ST, Leahy P, Alagramam KN, Megerian CA.

Otol Neurotol. 2007 Sep;28(6):834-41.


Identification and characterization of mouse cochlear stem cells.

Yerukhimovich MV, Bai L, Chen DH, Miller RH, Alagramam KN.

Dev Neurosci. 2007;29(3):251-60. Epub 2006 Oct 17.


Optimization of ribonucleic acid detection from archival Guinea pig temporal bone specimens.

Hall KL, Pitts DR, Anne S, Semaan MT, Alagramam KN, Megerian CA.

Otol Neurotol. 2007 Jan;28(1):116-23.


N-Acetyl L-cysteine does not protect against premature age-related hearing loss in C57BL/6J mice: a pilot study.

Davis RR, Kuo MW, Stanton SG, Canlon B, Krieg E, Alagramam KN.

Hear Res. 2007 Apr;226(1-2):203-8. Epub 2006 Aug 22.


A new spontaneous mutation in the mouse protocadherin 15 gene.

Zheng QY, Yu H, Washington JL 3rd, Kisley LB, Kikkawa YS, Pawlowski KS, Wright CG, Alagramam KN.

Hear Res. 2006 Sep;219(1-2):110-20. Epub 2006 Aug 2.


Inner ear proteomics of mouse models for deafness, a discovery strategy.

Zheng QY, Rozanas CR, Thalmann I, Chance MR, Alagramam KN.

Brain Res. 2006 May 26;1091(1):113-21. Epub 2006 Apr 5. Review.


Gene expression analysis of distinct populations of cells isolated from mouse and human inner ear FFPE tissue using laser capture microdissection--a technical report based on preliminary findings.

Pagedar NA, Wang W, Chen DH, Davis RR, Lopez I, Wright CG, Alagramam KN.

Brain Res. 2006 May 26;1091(1):289-99. Epub 2006 Mar 10.


Progression of inner ear pathology in Ames waltzer mice and the role of protocadherin 15 in hair cell development.

Pawlowski KS, Kikkawa YS, Wright CG, Alagramam KN.

J Assoc Res Otolaryngol. 2006 Jun;7(2):83-94. Epub 2006 Jan 12.


A quantitative survey of gravity receptor function in mutant mouse strains.

Jones SM, Johnson KR, Yu H, Erway LC, Alagramam KN, Pollak N, Jones TA.

J Assoc Res Otolaryngol. 2005 Dec;6(4):297-310.


The basic science of Meniere's disease and endolymphatic hydrops.

Semaan MT, Alagramam KN, Megerian CA.

Curr Opin Otolaryngol Head Neck Surg. 2005 Oct;13(5):301-7. Review.


Characterization of vestibular dysfunction in the mouse model for Usher syndrome 1F.

Alagramam KN, Stahl JS, Jones SM, Pawlowski KS, Wright CG.

J Assoc Res Otolaryngol. 2005 Jun;6(2):106-18. Epub 2005 Jun 10.


Interactions in the network of Usher syndrome type 1 proteins.

Adato A, Michel V, Kikkawa Y, Reiners J, Alagramam KN, Weil D, Yonekawa H, Wolfrum U, El-Amraoui A, Petit C.

Hum Mol Genet. 2005 Feb 1;14(3):347-56. Epub 2004 Dec 8.


Assessment of retinal structure and function in Ames waltzer mice.

Ball SL, Bardenstein D, Alagramam KN.

Invest Ophthalmol Vis Sci. 2003 Sep;44(9):3986-92. Erratum in: Invest Ophthalmol Vis Sci. 2004 Mar;45(3):775.


A new spontaneous mutation in the mouse Ames waltzer gene, Pcdh15.

Hampton LL, Wright CG, Alagramam KN, Battey JF, Noben-Trauth K.

Hear Res. 2003 Jun;180(1-2):67-75.


Mutations in the novel protocadherin PCDH15 cause Usher syndrome type 1F.

Alagramam KN, Yuan H, Kuehn MH, Murcia CL, Wayne S, Srisailpathy CR, Lowry RB, Knaus R, Van Laer L, Bernier FP, Schwartz S, Lee C, Morton CC, Mullins RF, Ramesh A, Van Camp G, Hageman GS, Woychik RP, Smith RJ.

Hum Mol Genet. 2001 Aug 1;10(16):1709-18. Erratum in: Hum Mol Genet 2001 Oct 15;10(22):2603. Hagemen GS [corrected to Hageman GS].


The mouse Ames waltzer hearing-loss mutant is caused by mutation of Pcdh15, a novel protocadherin gene.

Alagramam KN, Murcia CL, Kwon HY, Pawlowski KS, Wright CG, Woychik RP.

Nat Genet. 2001 Jan;27(1):99-102.


Neuroepithelial defects of the inner ear in a new allele of the mouse mutation Ames waltzer.

Alagramam KN, Zahorsky-Reeves J, Wright CG, Pawlowski KS, Erway LC, Stubbs L, Woychik RP.

Hear Res. 2000 Oct;148(1-2):181-91.


A new mouse insertional mutation that causes sensorineural deafness and vestibular defects.

Alagramam KN, Kwon HY, Cacheiro NL, Stubbs L, Wright CG, Erway LC, Woychik RP.

Genetics. 1999 Aug;152(4):1691-9.

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