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Items: 1 to 20 of 77

1.

Huntingtin fragments and SOD1 mutants form soluble oligomers in the cell.

Park YN, Zhao X, Norton M, Taylor JP, Eisenberg E, Greene LE.

PLoS One. 2012;7(6):e40329. doi: 10.1371/journal.pone.0040329. Epub 2012 Jun 29.

2.

Cellular toxicity of mutant SOD1 protein is linked to an easily soluble, non-aggregated form in vitro.

Brotherton TE, Li Y, Glass JD.

Neurobiol Dis. 2013 Jan;49:49-56. doi: 10.1016/j.nbd.2012.08.010. Epub 2012 Aug 25.

3.

Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction.

Goswami A, Dikshit P, Mishra A, Mulherkar S, Nukina N, Jana NR.

Biochem Biophys Res Commun. 2006 Mar 31;342(1):184-90. Epub 2006 Feb 3.

PMID:
16472774
4.

Inducible superoxide dismutase 1 aggregation in transgenic amyotrophic lateral sclerosis mouse fibroblasts.

Turner BJ, Lopes EC, Cheema SS.

J Cell Biochem. 2004 Apr 1;91(5):1074-84.

PMID:
15034941
5.

Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states.

Prudencio M, Borchelt DR.

Mol Neurodegener. 2011 Nov 17;6:77. doi: 10.1186/1750-1326-6-77.

6.

Small N-terminal mutant huntingtin fragments, but not wild type, are mainly present in monomeric form: Implications for pathogenesis.

Cong SY, Pepers BA, Roos RA, van Ommen GJ, Dorsman JC.

Exp Neurol. 2006 Jun;199(2):257-64. Epub 2005 Dec 27.

PMID:
16380118
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.

Polyanion binding accelerates the formation of stable and low-toxic aggregates of ALS-linked SOD1 mutant A4V.

Zhao D, Zhang S, Meng Y, Xiongwei D, Zhang D, Liang Y, Wang L, Liu C.

Proteins. 2014 Dec;82(12):3356-72. doi: 10.1002/prot.24691. Epub 2014 Oct 1.

PMID:
25220364
9.

Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice.

Xu G, Ayers JI, Roberts BL, Brown H, Fromholt S, Green C, Borchelt DR.

Hum Mol Genet. 2015 Feb 15;24(4):1019-35. doi: 10.1093/hmg/ddu517. Epub 2014 Oct 9.

10.

Structural changes to monomeric CuZn superoxide dismutase caused by the familial amyotrophic lateral sclerosis-associated mutation A4V.

Schmidlin T, Kennedy BK, Daggett V.

Biophys J. 2009 Sep 16;97(6):1709-18. doi: 10.1016/j.bpj.2009.06.043.

11.

Experimental mutagenesis of huntingtin to map cleavage sites: different outcomes in cell and mouse models.

Tebbenkamp AT, Xu G, Siemienski ZB, Janus C, Fromholt SE, Brown HH, Swing D, Tessarollo L, Borchelt DR.

J Huntingtons Dis. 2014;3(1):73-86. doi: 10.3233/JHD-130087.

PMID:
25062766
12.

Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues.

Watanabe M, Dykes-Hoberg M, Culotta VC, Price DL, Wong PC, Rothstein JD.

Neurobiol Dis. 2001 Dec;8(6):933-41.

PMID:
11741389
13.

Dysregulation of the proteasome increases the toxicity of ALS-linked mutant SOD1.

Kitamura A, Inada N, Kubota H, Matsumoto G, Kinjo M, Morimoto RI, Nagata K.

Genes Cells. 2014 Mar;19(3):209-24. doi: 10.1111/gtc.12125. Epub 2014 Jan 23.

14.

Immunodetection of disease-associated conformers of mutant cu/zn superoxide dismutase 1 selectively expressed in degenerating neurons in amyotrophic lateral sclerosis.

Sábado J, Casanovas A, Hernández S, Piedrafita L, Hereu M, Esquerda JE.

J Neuropathol Exp Neurol. 2013 Jul;72(7):646-61. doi: 10.1097/NEN.0b013e318297fd10.

PMID:
23771221
15.
16.

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic.

Takahashi T, Kikuchi S, Katada S, Nagai Y, Nishizawa M, Onodera O.

Hum Mol Genet. 2008 Feb 1;17(3):345-56. Epub 2007 Oct 18.

PMID:
17947294
17.
18.

Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments.

Li X, Wang CE, Huang S, Xu X, Li XJ, Li H, Li S.

Hum Mol Genet. 2010 Jun 15;19(12):2445-55. doi: 10.1093/hmg/ddq127. Epub 2010 Mar 30.

19.

Structural instability and Cu-dependent pro-oxidant activity acquired by the apo form of mutant SOD1 associated with amyotrophic lateral sclerosis.

Kitamura F, Fujimaki N, Okita W, Hiramatsu H, Takeuchi H.

Biochemistry. 2011 May 24;50(20):4242-50. doi: 10.1021/bi200338h. Epub 2011 May 2.

PMID:
21506602

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