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Items: 1 to 20 of 51

1.

Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin.

Waldron-Roby E, Ratovitski T, Wang X, Jiang M, Watkin E, Arbez N, Graham RK, Hayden MR, Hou Z, Mori S, Swing D, Pletnikov M, Duan W, Tessarollo L, Ross CA.

J Neurosci. 2012 Jan 4;32(1):183-93. doi: 10.1523/JNEUROSCI.1305-11.2012.

2.

Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative.

Tebbenkamp AT, Green C, Xu G, Denovan-Wright EM, Rising AC, Fromholt SE, Brown HH, Swing D, Mandel RJ, Tessarollo L, Borchelt DR.

Hum Mol Genet. 2011 Jul 15;20(14):2770-82. doi: 10.1093/hmg/ddr176. Epub 2011 Apr 22.

3.

Critical nucleus size for disease-related polyglutamine aggregation is repeat-length dependent.

Kar K, Jayaraman M, Sahoo B, Kodali R, Wetzel R.

Nat Struct Mol Biol. 2011 Mar;18(3):328-36. doi: 10.1038/nsmb.1992. Epub 2011 Feb 13.

4.

Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment.

Tebbenkamp AT, Swing D, Tessarollo L, Borchelt DR.

Hum Mol Genet. 2011 Apr 15;20(8):1633-42. doi: 10.1093/hmg/ddr040. Epub 2011 Feb 9.

5.

A compact beta model of huntingtin toxicity.

Zhang QC, Yeh TL, Leyva A, Frank LG, Miller J, Kim YE, Langen R, Finkbeiner S, Amzel ML, Ross CA, Poirier MA.

J Biol Chem. 2011 Mar 11;286(10):8188-96. doi: 10.1074/jbc.M110.192013. Epub 2011 Jan 5.

6.

Assessing mutant huntingtin fragment and polyglutamine aggregation by atomic force microscopy.

Burke KA, Godbey J, Legleiter J.

Methods. 2011 Mar;53(3):275-84. doi: 10.1016/j.ymeth.2010.12.028. Epub 2010 Dec 25.

PMID:
21187152
7.

Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool.

Olshina MA, Angley LM, Ramdzan YM, Tang J, Bailey MF, Hill AF, Hatters DM.

J Biol Chem. 2010 Jul 9;285(28):21807-16. doi: 10.1074/jbc.M109.084434. Epub 2010 May 5.

8.

Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo.

Legleiter J, Mitchell E, Lotz GP, Sapp E, Ng C, DiFiglia M, Thompson LM, Muchowski PJ.

J Biol Chem. 2010 May 7;285(19):14777-90. doi: 10.1074/jbc.M109.093708. Epub 2010 Mar 10.

9.
10.

Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice.

Gu X, Greiner ER, Mishra R, Kodali R, Osmand A, Finkbeiner S, Steffan JS, Thompson LM, Wetzel R, Yang XW.

Neuron. 2009 Dec 24;64(6):828-40. doi: 10.1016/j.neuron.2009.11.020.

11.

Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease.

Sathasivam K, Lane A, Legleiter J, Warley A, Woodman B, Finkbeiner S, Paganetti P, Muchowski PJ, Wilson S, Bates GP.

Hum Mol Genet. 2010 Jan 1;19(1):65-78. doi: 10.1093/hmg/ddp467.

12.

Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity.

Aiken CT, Steffan JS, Guerrero CM, Khashwji H, Lukacsovich T, Simmons D, Purcell JM, Menhaji K, Zhu YZ, Green K, Laferla F, Huang L, Thompson LM, Marsh JL.

J Biol Chem. 2009 Oct 23;284(43):29427-36. doi: 10.1074/jbc.M109.013193. Epub 2009 Aug 26.

13.

Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease.

Wacker JL, Huang SY, Steele AD, Aron R, Lotz GP, Nguyen Q, Giorgini F, Roberson ED, Lindquist S, Masliah E, Muchowski PJ.

J Neurosci. 2009 Jul 15;29(28):9104-14. doi: 10.1523/JNEUROSCI.2250-09.2009.

14.

Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism.

Thakur AK, Jayaraman M, Mishra R, Thakur M, Chellgren VM, Byeon IJ, Anjum DH, Kodali R, Creamer TP, Conway JF, Gronenborn AM, Wetzel R.

Nat Struct Mol Biol. 2009 Apr;16(4):380-9. doi: 10.1038/nsmb.1570. Epub 2009 Mar 8.

15.

Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells.

Ratovitski T, Gucek M, Jiang H, Chighladze E, Waldron E, D'Ambola J, Hou Z, Liang Y, Poirier MA, Hirschhorn RR, Graham R, Hayden MR, Cole RN, Ross CA.

J Biol Chem. 2009 Apr 17;284(16):10855-67. doi: 10.1074/jbc.M804813200. Epub 2009 Feb 9.

16.

The relationship between CAG repeat length and clinical progression in Huntington's disease.

Ravina B, Romer M, Constantinescu R, Biglan K, Brocht A, Kieburtz K, Shoulson I, McDermott MP.

Mov Disord. 2008 Jul 15;23(9):1223-7. doi: 10.1002/mds.21988.

PMID:
18512767
17.

Protein aggregation in the brain: the molecular basis for Alzheimer's and Parkinson's diseases.

Irvine GB, El-Agnaf OM, Shankar GM, Walsh DM.

Mol Med. 2008 Jul-Aug;14(7-8):451-64. doi: 10.2119/2007-00100.Irvine. Review.

18.

N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease.

Ratovitski T, Nakamura M, D'Ambola J, Chighladze E, Liang Y, Wang W, Graham R, Hayden MR, Borchelt DR, Hirschhorn RR, Ross CA.

Cell Cycle. 2007 Dec 1;6(23):2970-81.

PMID:
18156806
19.

Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease.

Weiss A, Klein C, Woodman B, Sathasivam K, Bibel M, RĂ©gulier E, Bates GP, Paganetti P.

J Neurochem. 2008 Feb;104(3):846-58. Epub 2007 Nov 6.

20.

Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide.

Haass C, Selkoe DJ.

Nat Rev Mol Cell Biol. 2007 Feb;8(2):101-12. Review.

PMID:
17245412

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