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Items: 1 to 20 of 60

1.

Role for a novel Usher protein complex in hair cell synaptic maturation.

Zallocchi M, Meehan DT, Delimont D, Rutledge J, Gratton MA, Flannery J, Cosgrove D.

PLoS One. 2012;7(2):e30573. doi: 10.1371/journal.pone.0030573. Epub 2012 Feb 17.

2.

Road to ruin: targeting proteins for degradation in the endoplasmic reticulum.

Smith MH, Ploegh HL, Weissman JS.

Science. 2011 Nov 25;334(6059):1086-90. doi: 10.1126/science.1209235. Review.

3.

Protein quality control in the ER: balancing the ubiquitin checkbook.

Claessen JH, Kundrat L, Ploegh HL.

Trends Cell Biol. 2012 Jan;22(1):22-32. doi: 10.1016/j.tcb.2011.09.010. Epub 2011 Nov 3. Review.

4.

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.

5.

TTC21B contributes both causal and modifying alleles across the ciliopathy spectrum.

Davis EE, Zhang Q, Liu Q, Diplas BH, Davey LM, Hartley J, Stoetzel C, Szymanska K, Ramaswami G, Logan CV, Muzny DM, Young AC, Wheeler DA, Cruz P, Morgan M, Lewis LR, Cherukuri P, Maskeri B, Hansen NF, Mullikin JC, Blakesley RW, Bouffard GG; NISC Comparative Sequencing Program, Gyapay G, Rieger S, Tönshoff B, Kern I, Soliman NA, Neuhaus TJ, Swoboda KJ, Kayserili H, Gallagher TE, Lewis RA, Bergmann C, Otto EA, Saunier S, Scambler PJ, Beales PL, Gleeson JG, Maher ER, Attié-Bitach T, Dollfus H, Johnson CA, Green ED, Gibbs RA, Hildebrandt F, Pierce EA, Katsanis N.

Nat Genet. 2011 Mar;43(3):189-96. doi: 10.1038/ng.756. Epub 2011 Jan 23.

6.

Using the cochlear microphonic as a tool to evaluate cochlear function in mouse models of hearing.

Cheatham MA, Naik K, Dallos P.

J Assoc Res Otolaryngol. 2011 Feb;12(1):113-25. doi: 10.1007/s10162-010-0240-5. Epub 2010 Oct 19.

7.

Hearing requires otoferlin-dependent efficient replenishment of synaptic vesicles in hair cells.

Pangrsic T, Lasarow L, Reuter K, Takago H, Schwander M, Riedel D, Frank T, Tarantino LM, Bailey JS, Strenzke N, Brose N, Müller U, Reisinger E, Moser T.

Nat Neurosci. 2010 Jul;13(7):869-76. doi: 10.1038/nn.2578. Epub 2010 Jun 20.

PMID:
20562868
8.

SnapShot: The unfolded protein response.

Wiseman RL, Haynes CM, Ron D.

Cell. 2010 Feb 19;140(4):590-590.e2. doi: 10.1016/j.cell.2010.02.006. No abstract available.

9.

Localization and expression of clarin-1, the Clrn1 gene product, in auditory hair cells and photoreceptors.

Zallocchi M, Meehan DT, Delimont D, Askew C, Garige S, Gratton MA, Rothermund-Franklin CA, Cosgrove D.

Hear Res. 2009 Sep;255(1-2):109-20. doi: 10.1016/j.heares.2009.06.006. Epub 2009 Jun 16.

10.

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.

11.

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.

12.

Tonotopic gradient in the developmental acquisition of sensory transduction in outer hair cells of the mouse cochlea.

Lelli A, Asai Y, Forge A, Holt JR, Géléoc GS.

J Neurophysiol. 2009 Jun;101(6):2961-73. doi: 10.1152/jn.00136.2009. Epub 2009 Apr 1.

13.

Prediction of disease-related mutations affecting protein localization.

Laurila K, Vihinen M.

BMC Genomics. 2009 Mar 23;10:122. doi: 10.1186/1471-2164-10-122.

14.

Tuning of synapse number, structure and function in the cochlea.

Meyer AC, Frank T, Khimich D, Hoch G, Riedel D, Chapochnikov NM, Yarin YM, Harke B, Hell SW, Egner A, Moser T.

Nat Neurosci. 2009 Apr;12(4):444-53. doi: 10.1038/nn.2293. Epub 2009 Mar 8.

PMID:
19270686
15.

Mouse models for human hereditary deafness.

Leibovici M, Safieddine S, Petit C.

Curr Top Dev Biol. 2008;84:385-429. doi: 10.1016/S0070-2153(08)00608-X. Review.

PMID:
19186249
16.

Update on Usher syndrome.

Saihan Z, Webster AR, Luxon L, Bitner-Glindzicz M.

Curr Opin Neurol. 2009 Feb;22(1):19-27. Review.

PMID:
19165952
17.

Trafficking of immature DeltaF508-CFTR to the plasma membrane and its detection by biotinylation.

Luo Y, McDonald K, Hanrahan JW.

Biochem J. 2009 Apr 1;419(1):211-9, 2 p following 219. doi: 10.1042/BJ20081869.

PMID:
19053947
18.

Retinal disease in Usher syndrome III caused by mutations in the clarin-1 gene.

Herrera W, Aleman TS, Cideciyan AV, Roman AJ, Banin E, Ben-Yosef T, Gardner LM, Sumaroka A, Windsor EA, Schwartz SB, Stone EM, Liu XZ, Kimberling WJ, Jacobson SG.

Invest Ophthalmol Vis Sci. 2008 Jun;49(6):2651-60. doi: 10.1167/iovs.07-1505. Epub 2008 Feb 15.

PMID:
18281613
19.

Mosaic complementation demonstrates a regulatory role for myosin VIIa in actin dynamics of stereocilia.

Prosser HM, Rzadzinska AK, Steel KP, Bradley A.

Mol Cell Biol. 2008 Mar;28(5):1702-12. Epub 2007 Dec 26.

20.

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