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Items: 1 to 20 of 154

1.

Polyglutamine expansion affects huntingtin conformation in multiple Huntington's disease models.

Daldin M, Fodale V, Cariulo C, Azzollini L, Verani M, Martufi P, Spiezia MC, Deguire SM, Cherubini M, Macdonald D, Weiss A, Bresciani A, Vonsattel JG, Petricca L, Marsh JL, Gines S, Santimone I, Marano M, Lashuel HA, Squitieri F, Caricasole A.

Sci Rep. 2017 Jul 11;7(1):5070. doi: 10.1038/s41598-017-05336-7.

2.

Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy.

Fodale V, Kegulian NC, Verani M, Cariulo C, Azzollini L, Petricca L, Daldin M, Boggio R, Padova A, Kuhn R, Pacifici R, Macdonald D, Schoenfeld RC, Park H, Isas JM, Langen R, Weiss A, Caricasole A.

PLoS One. 2014 Dec 2;9(12):e112262. doi: 10.1371/journal.pone.0112262. eCollection 2014. Erratum in: PLoS One. 2015;10(6):e0129986.

3.

Phosphorylation of huntingtin at residue T3 is decreased in Huntington's disease and modulates mutant huntingtin protein conformation.

Cariulo C, Azzollini L, Verani M, Martufi P, Boggio R, Chiki A, Deguire SM, Cherubini M, Gines S, Marsh JL, Conforti P, Cattaneo E, Santimone I, Squitieri F, Lashuel HA, Petricca L, Caricasole A.

Proc Natl Acad Sci U S A. 2017 Dec 12;114(50):E10809-E10818. doi: 10.1073/pnas.1705372114. Epub 2017 Nov 21.

5.

Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin.

Caron NS, Desmond CR, Xia J, Truant R.

Proc Natl Acad Sci U S A. 2013 Sep 3;110(36):14610-5. doi: 10.1073/pnas.1301342110. Epub 2013 Jul 29.

6.

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins.

Huang CC, Faber PW, Persichetti F, Mittal V, Vonsattel JP, MacDonald ME, Gusella JF.

Somat Cell Mol Genet. 1998 Jul;24(4):217-33.

PMID:
10410676
7.

Towards a transgenic model of Huntington's disease in a non-human primate.

Yang SH, Cheng PH, Banta H, Piotrowska-Nitsche K, Yang JJ, Cheng EC, Snyder B, Larkin K, Liu J, Orkin J, Fang ZH, Smith Y, Bachevalier J, Zola SM, Li SH, Li XJ, Chan AW.

Nature. 2008 Jun 12;453(7197):921-4. doi: 10.1038/nature06975. Epub 2008 May 18.

8.

Effects of overexpression of huntingtin proteins on mitochondrial integrity.

Wang H, Lim PJ, Karbowski M, Monteiro MJ.

Hum Mol Genet. 2009 Feb 15;18(4):737-52. doi: 10.1093/hmg/ddn404. Epub 2008 Nov 27.

9.

Polyglutamine tracts regulate beclin 1-dependent autophagy.

Ashkenazi A, Bento CF, Ricketts T, Vicinanza M, Siddiqi F, Pavel M, Squitieri F, Hardenberg MC, Imarisio S, Menzies FM, Rubinsztein DC.

Nature. 2017 May 4;545(7652):108-111. doi: 10.1038/nature22078. Epub 2017 Apr 26.

10.

Aggregation landscapes of Huntingtin exon 1 protein fragments and the critical repeat length for the onset of Huntington's disease.

Chen M, Wolynes PG.

Proc Natl Acad Sci U S A. 2017 Apr 25;114(17):4406-4411. doi: 10.1073/pnas.1702237114. Epub 2017 Apr 11.

11.

Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease.

Zhang H, Li Q, Graham RK, Slow E, Hayden MR, Bezprozvanny I.

Neurobiol Dis. 2008 Jul;31(1):80-8. doi: 10.1016/j.nbd.2008.03.010. Epub 2008 Apr 16.

12.

Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease.

Chan EY, Luthi-Carter R, Strand A, Solano SM, Hanson SA, DeJohn MM, Kooperberg C, Chase KO, DiFiglia M, Young AB, Leavitt BR, Cha JH, Aronin N, Hayden MR, Olson JM.

Hum Mol Genet. 2002 Aug 15;11(17):1939-51.

PMID:
12165556
13.

Folding Landscape of Mutant Huntingtin Exon1: Diffusible Multimers, Oligomers and Fibrils, and No Detectable Monomer.

Sahoo B, Arduini I, Drombosky KW, Kodali R, Sanders LH, Greenamyre JT, Wetzel R.

PLoS One. 2016 Jun 6;11(6):e0155747. doi: 10.1371/journal.pone.0155747. eCollection 2016.

14.

Quantification assays for total and polyglutamine-expanded huntingtin proteins.

Macdonald D, Tessari MA, Boogaard I, Smith M, Pulli K, Szynol A, Albertus F, Lamers MB, Dijkstra S, Kordt D, Reindl W, Herrmann F, McAllister G, Fischer DF, Munoz-Sanjuan I.

PLoS One. 2014 May 9;9(5):e96854. doi: 10.1371/journal.pone.0096854. eCollection 2014.

15.

Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington's disease.

Zhou X, Li G, Kaplan A, Gaschler MM, Zhang X, Hou Z, Jiang M, Zott R, Cremers S, Stockwell BR, Duan W.

Hum Mol Genet. 2018 May 1;27(9):1545-1555. doi: 10.1093/hmg/ddy061.

PMID:
29462355
16.

Conformational modulation mediated by polyglutamine expansion in CAG repeat expansion disease-associated proteins.

Verani M, Bustamante M, Martufi P, Daldin M, Cariulo C, Azzollini L, Fodale V, Puglisi F, Weiss A, Macdonald D, Petricca L, Caricasole A.

Biochem Biophys Res Commun. 2016 Sep 16;478(2):949-55. doi: 10.1016/j.bbrc.2016.08.057. Epub 2016 Aug 9.

PMID:
27520369
17.

Herp Promotes Degradation of Mutant Huntingtin: Involvement of the Proteasome and Molecular Chaperones.

Luo H, Cao L, Liang X, Du A, Peng T, Li H.

Mol Neurobiol. 2018 Oct;55(10):7652-7668. doi: 10.1007/s12035-018-0900-8. Epub 2018 Feb 12.

PMID:
29430620
18.

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

Wellington CL, Ellerby LM, Gutekunst CA, Rogers D, Warby S, Graham RK, Loubser O, van Raamsdonk J, Singaraja R, Yang YZ, Gafni J, Bredesen D, Hersch SM, Leavitt BR, Roy S, Nicholson DW, Hayden MR.

J Neurosci. 2002 Sep 15;22(18):7862-72.

19.

Pathogenic Huntington Alters BMP Signaling and Synaptic Growth through Local Disruptions of Endosomal Compartments.

Akbergenova Y, Littleton JT.

J Neurosci. 2017 Mar 22;37(12):3425-3439. doi: 10.1523/JNEUROSCI.2752-16.2017. Epub 2017 Feb 24.

20.

PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD).

Ratovitski T, Arbez N, Stewart JC, Chighladze E, Ross CA.

Cell Cycle. 2015;14(11):1716-29. doi: 10.1080/15384101.2015.1033595.

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