Davies JE - Search Results

1

Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres.

Trollet C, Anvar SY, Venema A, Hargreaves IP, Foster K, Vignaud A, Ferry A, Negroni E, Hourde C, Baraibar MA, 't Hoen PA, Davies JE, Rubinsztein DC, Heales SJ, Mouly V, van der Maarel SM, Butler-Browne G, Raz V, Dickson G. Trollet C, et al. Among authors: davies je. Hum Mol Genet. 2010 Jun 1;19(11):2191-207. doi: 10.1093/hmg/ddq098. Epub 2010 Mar 5. Hum Mol Genet. 2010. PMID: 20207626

2

Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy.

Davies JE, Sarkar S, Rubinsztein DC. Davies JE, et al. Hum Mol Genet. 2006 Jan 1;15(1):23-31. doi: 10.1093/hmg/ddi422. Epub 2005 Nov 25. Hum Mol Genet. 2006. PMID: 16311254

3

Wild-type PABPN1 is anti-apoptotic and reduces toxicity of the oculopharyngeal muscular dystrophy mutation.

Davies JE, Sarkar S, Rubinsztein DC. Davies JE, et al. Hum Mol Genet. 2008 Apr 15;17(8):1097-108. doi: 10.1093/hmg/ddm382. Epub 2008 Jan 4. Hum Mol Genet. 2008. PMID: 18178579

4

Over-expression of BCL2 rescues muscle weakness in a mouse model of oculopharyngeal muscular dystrophy.

Davies JE, Rubinsztein DC. Davies JE, et al. Hum Mol Genet. 2011 Mar 15;20(6):1154-63. doi: 10.1093/hmg/ddq559. Epub 2011 Jan 3. Hum Mol Genet. 2011. PMID: 21199860 Free PMC article.

5

Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice.

Davies JE, Wang L, Garcia-Oroz L, Cook LJ, Vacher C, O'Donovan DG, Rubinsztein DC. Davies JE, et al. Nat Med. 2005 Jun;11(6):672-7. doi: 10.1038/nm1242. Epub 2005 May 1. Nat Med. 2005. PMID: 15864313

6

Deleterious and protective properties of an aggregate-prone protein with a polyalanine expansion.

Berger Z, Davies JE, Luo S, Pasco MY, Majoul I, O'Kane CJ, Rubinsztein DC. Berger Z, et al. Among authors: davies je. Hum Mol Genet. 2006 Feb 1;15(3):453-65. doi: 10.1093/hmg/ddi460. Epub 2005 Dec 21. Hum Mol Genet. 2006. PMID: 16371423

7

Oculopharyngeal muscular dystrophy: potential therapies for an aggregate-associated disorder.

Davies JE, Berger Z, Rubinsztein DC. Davies JE, et al. Int J Biochem Cell Biol. 2006;38(9):1457-62. doi: 10.1016/j.biocel.2006.01.016. Epub 2006 Feb 28. Int J Biochem Cell Biol. 2006. PMID: 16530457 Review.

8

Cystamine suppresses polyalanine toxicity in a mouse model of oculopharyngeal muscular dystrophy.

Davies JE, Rose C, Sarkar S, Rubinsztein DC. Davies JE, et al. Sci Transl Med. 2010 Jun 2;2(34):34ra40. doi: 10.1126/scitranslmed.3000723. Sci Transl Med. 2010. PMID: 20519718

9

Polyalanine and polyserine frameshift products in Huntington's disease.

Davies JE, Rubinsztein DC. Davies JE, et al. J Med Genet. 2006 Nov;43(11):893-6. doi: 10.1136/jmg.2006.044222. Epub 2006 Jun 26. J Med Genet. 2006. PMID: 16801344 Free PMC article.

10

The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias.

Davies JE, Sarkar S, Rubinsztein DC. Davies JE, et al. BMC Biochem. 2007 Nov 22;8 Suppl 1(Suppl 1):S2. doi: 10.1186/1471-2091-8-S1-S2. BMC Biochem. 2007. PMID: 18047739 Free PMC article. Review.

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