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Items: 1 to 20 of 36

1.

An examination of alpha B-crystallin as a modifier of SOD1 aggregate pathology and toxicity in models of familial amyotrophic lateral sclerosis.

Karch CM, Borchelt DR.

J Neurochem. 2010 Jun;113(5):1092-100. doi: 10.1111/j.1471-4159.2010.06572.x. Epub 2010 Jan 7.

2.

Wild-type Cu/Zn superoxide dismutase (SOD1) does not facilitate, but impedes the formation of protein aggregates of amyotrophic lateral sclerosis causing mutant SOD1.

Witan H, Gorlovoy P, Kaya AM, Koziollek-Drechsler I, Neumann H, Behl C, Clement AM.

Neurobiol Dis. 2009 Nov;36(2):331-42. doi: 10.1016/j.nbd.2009.07.024. Epub 2009 Aug 4.

PMID:
19660548
3.

Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease.

Prudencio M, Hart PJ, Borchelt DR, Andersen PM.

Hum Mol Genet. 2009 Sep 1;18(17):3217-26. doi: 10.1093/hmg/ddp260. Epub 2009 May 30.

4.

Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS.

Karch CM, Prudencio M, Winkler DD, Hart PJ, Borchelt DR.

Proc Natl Acad Sci U S A. 2009 May 12;106(19):7774-9. doi: 10.1073/pnas.0902505106. Epub 2009 Apr 30.

5.

Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse.

Wang L, Deng HX, Grisotti G, Zhai H, Siddique T, Roos RP.

Hum Mol Genet. 2009 May 1;18(9):1642-51. doi: 10.1093/hmg/ddp085. Epub 2009 Feb 19.

6.

Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme.

Prudencio M, Durazo A, Whitelegge JP, Borchelt DR.

J Neurochem. 2009 Feb;108(4):1009-18. doi: 10.1111/j.1471-4159.2008.05839.x. Epub 2008 Dec 11.

7.

Initiation and elongation in fibrillation of ALS-linked superoxide dismutase.

Chattopadhyay M, Durazo A, Sohn SH, Strong CD, Gralla EB, Whitelegge JP, Valentine JS.

Proc Natl Acad Sci U S A. 2008 Dec 2;105(48):18663-8. doi: 10.1073/pnas.0807058105. Epub 2008 Nov 20.

8.

Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival.

Wang Q, Johnson JL, Agar NY, Agar JN.

PLoS Biol. 2008 Jul 29;6(7):e170. doi: 10.1371/journal.pbio.0060170.

9.

Molecular dissection of ALS-associated toxicity of SOD1 in transgenic mice using an exon-fusion approach.

Deng HX, Jiang H, Fu R, Zhai H, Shi Y, Liu E, Hirano M, Dal Canto MC, Siddique T.

Hum Mol Genet. 2008 Aug 1;17(15):2310-9. doi: 10.1093/hmg/ddn131. Epub 2008 Apr 18. Erratum in: Hum Mol Genet. 2009 Feb 1;18(3):594. Han-Xiang, Deng [corrected to Deng, Han-Xiang]; Hujun, Jiang [corrected to Jiang, Hujun]; Ronggen, Fu [corrected to Fu, Ronggen]; Hong, Zhai [corrected to Zhai, Hong]; Yong, Shi [corrected to Shi, Yong]; Erdong, Liu [corrected to Liu, Erdong]; Makito, Hirano [corrected to Hirano, Makito]; Mauro, C Dal Canto [corrected to Dal Canto, Mauro C]; Teepu, Siddique [corrected to Siddique, Teepu].

10.

A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis.

Karch CM, Borchelt DR.

J Biol Chem. 2008 May 16;283(20):13528-37. doi: 10.1074/jbc.M800564200. Epub 2008 Mar 3.

11.

Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice.

Jaarsma D, Teuling E, Haasdijk ED, De Zeeuw CI, Hoogenraad CC.

J Neurosci. 2008 Feb 27;28(9):2075-88. doi: 10.1523/JNEUROSCI.5258-07.2008.

12.

Heterodimer formation of wild-type and amyotrophic lateral sclerosis-causing mutant Cu/Zn-superoxide dismutase induces toxicity independent of protein aggregation.

Witan H, Kern A, Koziollek-Drechsler I, Wade R, Behl C, Clement AM.

Hum Mol Genet. 2008 May 15;17(10):1373-85. doi: 10.1093/hmg/ddn025. Epub 2008 Jan 22.

13.

Protein aggregation processes: In search of the mechanism.

Frieden C.

Protein Sci. 2007 Nov;16(11):2334-44. Review.

14.

Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models.

Zetterström P, Stewart HG, Bergemalm D, Jonsson PA, Graffmo KS, Andersen PM, Brännström T, Oliveberg M, Marklund SL.

Proc Natl Acad Sci U S A. 2007 Aug 28;104(35):14157-62. Epub 2007 Aug 21.

15.

Wild-type superoxide dismutase acquires binding and toxic properties of ALS-linked mutant forms through oxidation.

Ezzi SA, Urushitani M, Julien JP.

J Neurochem. 2007 Jul;102(1):170-8. Epub 2007 Mar 29.

16.

Disease-associated mutations at copper ligand histidine residues of superoxide dismutase 1 diminish the binding of copper and compromise dimer stability.

Wang J, Caruano-Yzermans A, Rodriguez A, Scheurmann JP, Slunt HH, Cao X, Gitlin J, Hart PJ, Borchelt DR.

J Biol Chem. 2007 Jan 5;282(1):345-52. Epub 2006 Nov 8.

17.

Insoluble mutant SOD1 is partly oligoubiquitinated in amyotrophic lateral sclerosis mice.

Basso M, Massignan T, Samengo G, Cheroni C, De Biasi S, Salmona M, Bendotti C, Bonetto V.

J Biol Chem. 2006 Nov 3;281(44):33325-35. Epub 2006 Aug 30.

18.

Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria.

Deng HX, Shi Y, Furukawa Y, Zhai H, Fu R, Liu E, Gorrie GH, Khan MS, Hung WY, Bigio EH, Lukas T, Dal Canto MC, O'Halloran TV, Siddique T.

Proc Natl Acad Sci U S A. 2006 May 2;103(18):7142-7. Epub 2006 Apr 24.

19.

Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models.

Jonsson PA, Graffmo KS, Andersen PM, Brännström T, Lindberg M, Oliveberg M, Marklund SL.

Brain. 2006 Feb;129(Pt 2):451-64. Epub 2005 Dec 5.

20.

Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA.

Wang J, Xu G, Slunt HH, Gonzales V, Coonfield M, Fromholt D, Copeland NG, Jenkins NA, Borchelt DR.

Neurobiol Dis. 2005 Dec;20(3):943-52. Epub 2005 Jul 19.

PMID:
16046140
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