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Items: 7

1.

Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease.

Lu XH, Mattis VB, Wang N, Al-Ramahi I, van den Berg N, Fratantoni SA, Waldvogel H, Greiner E, Osmand A, Elzein K, Xiao J, Dijkstra S, de Pril R, Vinters HV, Faull R, Signer E, Kwak S, Marugan JJ, Botas J, Fischer DF, Svendsen CN, Munoz-Sanjuan I, Yang XW.

Sci Transl Med. 2014 Dec 24;6(268):268ra178. doi: 10.1126/scitranslmed.3010523.

PMID:
25540325
2.

Modest proteasomal inhibition by aberrant ubiquitin exacerbates aggregate formation in a Huntington disease mouse model.

de Pril R, Hobo B, van Tijn P, Roos RA, van Leeuwen FW, Fischer DF.

Mol Cell Neurosci. 2010 Mar;43(3):281-6. doi: 10.1016/j.mcn.2009.12.001. Epub 2009 Dec 18.

PMID:
20005957
3.

Ubiquitin-conjugating enzyme E2-25K increases aggregate formation and cell death in polyglutamine diseases.

de Pril R, Fischer DF, Roos RA, van Leeuwen FW.

Mol Cell Neurosci. 2007 Jan;34(1):10-9. Epub 2006 Nov 7.

PMID:
17092742
4.

Conformational diseases: an umbrella for various neurological disorders with an impaired ubiquitin-proteasome system.

de Pril R, Fischer DF, van Leeuwen FW.

Neurobiol Aging. 2006 Apr;27(4):515-23. Epub 2005 Oct 13. Review.

PMID:
16226348
5.

Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases.

de Pril R, Fischer DF, Maat-Schieman ML, Hobo B, de Vos RA, Brunt ER, Hol EM, Roos RA, van Leeuwen FW.

Hum Mol Genet. 2004 Aug 15;13(16):1803-13. Epub 2004 Jun 15.

PMID:
15198995
6.

Repair of UV lesions in silenced chromatin provides in vivo evidence for a compact chromatin structure.

Livingstone-Zatchej M, Marcionelli R, Möller K, de Pril R, Thoma F.

J Biol Chem. 2003 Sep 26;278(39):37471-9. Epub 2003 Jul 25.

7.

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