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Items: 1 to 20 of 105

1.

Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy.

Barbezier N, Chartier A, Bidet Y, Buttstedt A, Voisset C, Galons H, Blondel M, Schwarz E, Simonelig M.

EMBO Mol Med. 2011 Jan;3(1):35-49. doi: 10.1002/emmm.201000109.

2.

HnRNP A1 and A/B interaction with PABPN1 in oculopharyngeal muscular dystrophy.

Fan X, Messaed C, Dion P, Laganiere J, Brais B, Karpati G, Rouleau GA.

Can J Neurol Sci. 2003 Aug;30(3):244-51.

PMID:
12945950
3.

A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1.

Chartier A, Benoit B, Simonelig M.

EMBO J. 2006 May 17;25(10):2253-62. Epub 2006 Apr 27.

4.

Prevention of oculopharyngeal muscular dystrophy by muscular expression of Llama single-chain intrabodies in vivo.

Chartier A, Raz V, Sterrenburg E, Verrips CT, van der Maarel SM, Simonelig M.

Hum Mol Genet. 2009 May 15;18(10):1849-59. doi: 10.1093/hmg/ddp101. Epub 2009 Mar 3.

PMID:
19258344
5.

The various facets of the protein-folding activity of the ribosome.

Voisset C, Saupe SJ, Blondel M.

Biotechnol J. 2011 Jun;6(6):668-73. doi: 10.1002/biot.201100021. Epub 2011 May 12. Review.

PMID:
21567961
6.

Protein folding activity of the ribosome (PFAR) -- a target for antiprion compounds.

Banerjee D, Sanyal S.

Viruses. 2014 Oct 23;6(10):3907-24. doi: 10.3390/v6103907. Review.

7.

Prevention of oculopharyngeal muscular dystrophy-associated aggregation of nuclear polyA-binding protein with a single-domain intracellular antibody.

Verheesen P, de Kluijver A, van Koningsbruggen S, de Brij M, de Haard HJ, van Ommen GJ, van der Maarel SM, Verrips CT.

Hum Mol Genet. 2006 Jan 1;15(1):105-11. Epub 2005 Nov 30.

PMID:
16319127
8.

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

Davies JE, Berger Z, Rubinsztein DC.

Int J Biochem Cell Biol. 2006;38(9):1457-62. Epub 2006 Feb 28. Review.

PMID:
16530457
9.

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

Davies JE, Sarkar S, Rubinsztein DC.

Hum Mol Genet. 2006 Jan 1;15(1):23-31. Epub 2005 Nov 25.

PMID:
16311254
10.

Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 protein.

Raz V, Routledge S, Venema A, Buijze H, van der Wal E, Anvar S, Straasheijm KR, Klooster R, Antoniou M, van der Maarel SM.

Am J Pathol. 2011 Oct;179(4):1988-2000. doi: 10.1016/j.ajpath.2011.06.044. Epub 2011 Aug 18.

11.

Soluble expanded PABPN1 promotes cell death in oculopharyngeal muscular dystrophy.

Messaed C, Dion PA, Abu-Baker A, Rochefort D, Laganiere J, Brais B, Rouleau GA.

Neurobiol Dis. 2007 Jun;26(3):546-57. Epub 2007 Feb 15.

PMID:
17418585
12.
13.

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

Davies JE, Sarkar S, Rubinsztein DC.

Hum Mol Genet. 2008 Apr 15;17(8):1097-108. doi: 10.1093/hmg/ddm382. Epub 2008 Jan 4.

PMID:
18178579
14.

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.

Hum Mol Genet. 2010 Jun 1;19(11):2191-207. doi: 10.1093/hmg/ddq098. Epub 2010 Mar 5.

PMID:
20207626
15.

PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions.

Corbeil-Girard LP, Klein AF, Sasseville AM, Lavoie H, Dicaire MJ, Saint-Denis A, Pagé M, Duranceau A, Codère F, Bouchard JP, Karpati G, Rouleau GA, Massie B, Langelier Y, Brais B.

Neurobiol Dis. 2005 Apr;18(3):551-67.

PMID:
15755682
16.

Polyalanine-independent conformational conversion of nuclear poly(A)-binding protein 1 (PABPN1).

Winter R, Kühn U, Hause G, Schwarz E.

J Biol Chem. 2012 Jun 29;287(27):22662-71. doi: 10.1074/jbc.M112.362327. Epub 2012 May 8.

17.

Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy.

Raz V, Sterrenburg E, Routledge S, Venema A, van der Sluijs BM, Trollet C, Dickson G, van Engelen BG, van der Maarel SM, Antoniou MN.

BMC Neurol. 2013 Jul 1;13:70. doi: 10.1186/1471-2377-13-70.

18.

Nuclear speckles are involved in nuclear aggregation of PABPN1 and in the pathophysiology of oculopharyngeal muscular dystrophy.

Bengoechea R, Tapia O, Casafont I, Berciano J, Lafarga M, Berciano MT.

Neurobiol Dis. 2012 Apr;46(1):118-29. doi: 10.1016/j.nbd.2011.12.052. Epub 2012 Jan 10.

PMID:
22249111
19.

Study of a Taiwanese family with oculopharyngeal muscular dystrophy.

Kuo HC, Chen CM, Lee-Chen GJ, Hu FJ, Chu CC, Liou CW, Huang CC.

J Neurol Sci. 2009 Mar 15;278(1-2):21-4. doi: 10.1016/j.jns.2008.11.001. Epub 2008 Dec 20.

PMID:
19101703
20.

Protein folding activity of ribosomal RNA is a selective target of two unrelated antiprion drugs.

Tribouillard-Tanvier D, Dos Reis S, Gug F, Voisset C, Béringue V, Sabate R, Kikovska E, Talarek N, Bach S, Huang C, Desban N, Saupe SJ, Supattapone S, Thuret JY, Chédin S, Vilette D, Galons H, Sanyal S, Blondel M.

PLoS One. 2008 May 14;3(5):e2174. doi: 10.1371/journal.pone.0002174.

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