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Items: 1 to 20 of 108

1.

Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease.

Weiss A, Abramowski D, Bibel M, Bodner R, Chopra V, DiFiglia M, Fox J, Kegel K, Klein C, Grueninger S, Hersch S, Housman D, RĂ©gulier E, Rosas HD, Stefani M, Zeitlin S, Bilbe G, Paganetti P.

Anal Biochem. 2009 Dec 1;395(1):8-15. doi: 10.1016/j.ab.2009.08.001. Epub 2009 Aug 6.

PMID:
19664996
2.

Huntingtin aggregation kinetics and their pathological role in a Drosophila Huntington's disease model.

Weiss KR, Kimura Y, Lee WC, Littleton JT.

Genetics. 2012 Feb;190(2):581-600. doi: 10.1534/genetics.111.133710. Epub 2011 Nov 17.

3.

Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat.

Clemens LE, Weber JJ, Wlodkowski TT, Yu-Taeger L, Michaud M, Calaminus C, Eckert SH, Gaca J, Weiss A, Magg JC, Jansson EK, Eckert GP, Pichler BJ, Bordet T, Pruss RM, Riess O, Nguyen HP.

Brain. 2015 Dec;138(Pt 12):3632-53. doi: 10.1093/brain/awv290. Epub 2015 Oct 21.

PMID:
26490331
4.

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.

5.

Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging.

Marcellin D, Abramowski D, Young D, Richter J, Weiss A, Marcel A, Maassen J, Kauffmann M, Bibel M, Shimshek DR, Faull RL, Bates GP, Kuhn RR, Van der Putten PH, Schmid P, Lotz GP.

PLoS One. 2012;7(9):e44457. doi: 10.1371/journal.pone.0044457. Epub 2012 Sep 12.

6.

Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease.

Valencia A, Reeves PB, Sapp E, Li X, Alexander J, Kegel KB, Chase K, Aronin N, DiFiglia M.

J Neurosci Res. 2010 Jan;88(1):179-90. doi: 10.1002/jnr.22184.

PMID:
19642201
7.

Mutant huntingtin promotes the fibrillogenesis of wild-type huntingtin: a potential mechanism for loss of huntingtin function in Huntington's disease.

Busch A, Engemann S, Lurz R, Okazawa H, Lehrach H, Wanker EE.

J Biol Chem. 2003 Oct 17;278(42):41452-61. Epub 2003 Jul 29.

8.

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
9.

TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease.

Baldo B, Paganetti P, Grueninger S, Marcellin D, Kaltenbach LS, Lo DC, Semmelroth M, Zivanovic A, Abramowski D, Smith D, Lotz GP, Bates GP, Weiss A.

Chem Biol. 2012 Feb 24;19(2):264-75. doi: 10.1016/j.chembiol.2011.12.020.

10.

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.

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.

Microtiter plate quantification of mutant and wild-type huntingtin normalized to cell count.

Weiss A, Grueninger S, Abramowski D, Giorgio FP, Lopatin MM, Rosas HD, Hersch S, Paganetti P.

Anal Biochem. 2011 Mar 15;410(2):304-6. doi: 10.1016/j.ab.2010.11.044. Epub 2010 Dec 4.

PMID:
21134349
13.

Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum.

Grondin R, Kaytor MD, Ai Y, Nelson PT, Thakker DR, Heisel J, Weatherspoon MR, Blum JL, Burright EN, Zhang Z, Kaemmerer WF.

Brain. 2012 Apr;135(Pt 4):1197-209. doi: 10.1093/brain/awr333. Epub 2012 Jan 16.

15.

Preventing formation of toxic N-terminal huntingtin fragments through antisense oligonucleotide-mediated protein modification.

Evers MM, Tran HD, Zalachoras I, Meijer OC, den Dunnen JT, van Ommen GJ, Aartsma-Rus A, van Roon-Mom WM.

Nucleic Acid Ther. 2014 Feb;24(1):4-12. doi: 10.1089/nat.2013.0452. Epub 2013 Dec 31.

PMID:
24380395
16.

Arfaptin 2 regulates the aggregation of mutant huntingtin protein.

Peters PJ, Ning K, Palacios F, Boshans RL, Kazantsev A, Thompson LM, Woodman B, Bates GP, D'Souza-Schorey C.

Nat Cell Biol. 2002 Mar;4(3):240-5.

PMID:
11854752
17.

Mechanisms of copper ion mediated Huntington's disease progression.

Fox JH, Kama JA, Lieberman G, Chopra R, Dorsey K, Chopra V, Volitakis I, Cherny RA, Bush AI, Hersch S.

PLoS One. 2007 Mar 28;2(3):e334.

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.

Interaction of the nuclear matrix protein NAKAP with HypA and huntingtin: implications for nuclear toxicity in Huntington's disease pathogenesis.

Sayer JA, Manczak M, Akileswaran L, Reddy PH, Coghlan VM.

Neuromolecular Med. 2005;7(4):297-310.

PMID:
16391387
20.

Lack of huntingtin-associated protein-1 causes neuronal death resembling hypothalamic degeneration in Huntington's disease.

Li SH, Yu ZX, Li CL, Nguyen HP, Zhou YX, Deng C, Li XJ.

J Neurosci. 2003 Jul 30;23(17):6956-64.

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