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Items: 1 to 20 of 121

1.

The R109H variant of fascin-2, a developmentally regulated actin crosslinker in hair-cell stereocilia, underlies early-onset hearing loss of DBA/2J mice.

Shin JB, Longo-Guess CM, Gagnon LH, Saylor KW, Dumont RA, Spinelli KJ, Pagana JM, Wilmarth PA, David LL, Gillespie PG, Johnson KR.

J Neurosci. 2010 Jul 21;30(29):9683-94. doi: 10.1523/JNEUROSCI.1541-10.2010.

2.

A QTL on Chr 5 modifies hearing loss associated with the fascin-2 variant of DBA/2J mice.

Johnson KR, Longo-Guess CM, Gagnon LH.

Mamm Genome. 2015 Aug;26(7-8):338-47. doi: 10.1007/s00335-015-9574-y. Epub 2015 Jun 20.

3.

Quantitative trait loci on chromosome 5 for susceptibility to frequency-specific effects on hearing in DBA/2J mice.

Suzuki S, Ishikawa M, Ueda T, Ohshiba Y, Miyasaka Y, Okumura K, Yokohama M, Taya C, Matsuoka K, Kikkawa Y.

Exp Anim. 2015;64(3):241-51. doi: 10.1538/expanim.14-0110. Epub 2015 Mar 10.

4.

β-Actin and fascin-2 cooperate to maintain stereocilia length.

Perrin BJ, Strandjord DM, Narayanan P, Henderson DM, Johnson KR, Ervasti JM.

J Neurosci. 2013 May 8;33(19):8114-21. doi: 10.1523/JNEUROSCI.0238-13.2013.

5.

Plastin 1 widens stereocilia by transforming actin filament packing from hexagonal to liquid.

Krey JF, Krystofiak ES, Dumont RA, Vijayakumar S, Choi D, Rivero F, Kachar B, Jones SM, Barr-Gillespie PG.

J Cell Biol. 2016 Nov 21;215(4):467-482. Epub 2016 Nov 3.

6.

A new Atp2b2 deafwaddler allele, dfw(i5), interacts strongly with Cdh23 and other auditory modifiers.

Watson CJ, Tempel BL.

Hear Res. 2013 Oct;304:41-8. doi: 10.1016/j.heares.2013.06.003. Epub 2013 Jun 18.

7.

A short splice form of Xin-actin binding repeat containing 2 (XIRP2) lacking the Xin repeats is required for maintenance of stereocilia morphology and hearing function.

Francis SP, Krey JF, Krystofiak ES, Cui R, Nanda S, Xu W, Kachar B, Barr-Gillespie PG, Shin JB.

J Neurosci. 2015 Feb 4;35(5):1999-2014. doi: 10.1523/JNEUROSCI.3449-14.2015.

8.

Attenuation of hearing loss in DBA/2J mice by anti-apoptotic treatment.

Yang L, Zhang H, Han X, Zhao X, Hu F, Li P, Xie G, Gao L, Cheng L, Song X, Han F.

Hear Res. 2015 Sep;327:109-16. doi: 10.1016/j.heares.2015.05.006. Epub 2015 May 21.

PMID:
26003529
9.

Absence of plastin 1 causes abnormal maintenance of hair cell stereocilia and a moderate form of hearing loss in mice.

Taylor R, Bullen A, Johnson SL, Grimm-Günter EM, Rivero F, Marcotti W, Forge A, Daudet N.

Hum Mol Genet. 2015 Jan 1;24(1):37-49. doi: 10.1093/hmg/ddu417. Epub 2014 Aug 14.

10.

A locus on distal chromosome 11 (ahl8) and its interaction with Cdh23 ahl underlie the early onset, age-related hearing loss of DBA/2J mice.

Johnson KR, Longo-Guess C, Gagnon LH, Yu H, Zheng QY.

Genomics. 2008 Oct;92(4):219-25. doi: 10.1016/j.ygeno.2008.06.007. Epub 2008 Aug 15.

11.

Mutations in Cdh23, encoding a new type of cadherin, cause stereocilia disorganization in waltzer, the mouse model for Usher syndrome type 1D.

Di Palma F, Holme RH, Bryda EC, Belyantseva IA, Pellegrino R, Kachar B, Steel KP, Noben-Trauth K.

Nat Genet. 2001 Jan;27(1):103-7.

PMID:
11138008
12.

Correlation of expression of the actin filament-bundling protein espin with stereociliary bundle formation in the developing inner ear.

Li H, Liu H, Balt S, Mann S, Corrales CE, Heller S.

J Comp Neurol. 2004 Jan 1;468(1):125-34.

PMID:
14648695
13.

The chloride intracellular channel protein CLIC5 is expressed at high levels in hair cell stereocilia and is essential for normal inner ear function.

Gagnon LH, Longo-Guess CM, Berryman M, Shin JB, Saylor KW, Yu H, Gillespie PG, Johnson KR.

J Neurosci. 2006 Oct 4;26(40):10188-98.

14.

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.

15.

Compound heterozygosity of the functionally null Cdh23(v-ngt) and hypomorphic Cdh23(ahl) alleles leads to early-onset progressive hearing loss in mice.

Miyasaka Y, Suzuki S, Ohshiba Y, Watanabe K, Sagara Y, Yasuda SP, Matsuoka K, Shitara H, Yonekawa H, Kominami R, Kikkawa Y.

Exp Anim. 2013;62(4):333-46.

16.

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.

17.

Characterization of human retinal fascin gene (FSCN2) at 17q25: close physical linkage of fascin and cytoplasmic actin genes.

Tubb BE, Bardien-Kruger S, Kashork CD, Shaffer LG, Ramagli LS, Xu J, Siciliano MJ, Bryan J.

Genomics. 2000 Apr 15;65(2):146-56.

PMID:
10783262
18.

Disruption of SorCS2 reveals differences in the regulation of stereociliary bundle formation between hair cell types in the inner ear.

Forge A, Taylor RR, Dawson SJ, Lovett M, Jagger DJ.

PLoS Genet. 2017 Mar 27;13(3):e1006692. doi: 10.1371/journal.pgen.1006692. eCollection 2017 Mar.

19.

Heterozygous mutation of Ush1g/Sans in mice causes early-onset progressive hearing loss, which is recovered by reconstituting the strain-specific mutation in Cdh23.

Miyasaka Y, Shitara H, Suzuki S, Yoshimoto S, Seki Y, Ohshiba Y, Okumura K, Taya C, Tokano H, Kitamura K, Takada T, Hibino H, Shiroishi T, Kominami R, Yonekawa H, Kikkawa Y.

Hum Mol Genet. 2016 May 15;25(10):2045-2059. Epub 2016 Mar 2.

PMID:
26936824
20.

Fascin 2b is a component of stereocilia that lengthens actin-based protrusions.

Chou SW, Hwang P, Gomez G, Fernando CA, West MC, Pollock LM, Lin-Jones J, Burnside B, McDermott BM Jr.

PLoS One. 2011;6(4):e14807. doi: 10.1371/journal.pone.0014807. Epub 2011 Apr 26.

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