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Items: 1 to 20 of 204


Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice.

Wang L, Gutmann DH, Roos RP.

Hum Mol Genet. 2011 Jan 15;20(2):286-93. doi: 10.1093/hmg/ddq463. Epub 2010 Oct 20.


The effect of mutant SOD1 dismutase activity on non-cell autonomous degeneration in familial amyotrophic lateral sclerosis.

Wang L, Sharma K, Grisotti G, Roos RP.

Neurobiol Dis. 2009 Aug;35(2):234-40. doi: 10.1016/j.nbd.2009.05.002. Epub 2009 May 12.


Selective knockdown of mutant SOD1 in Schwann cells ameliorates disease in G85R mutant SOD1 transgenic mice.

Wang L, Pytel P, Feltri ML, Wrabetz L, Roos RP.

Neurobiol Dis. 2012 Oct;48(1):52-7. doi: 10.1016/j.nbd.2012.05.014. Epub 2012 Jun 2. Erratum in: Neurobiol Dis. 2013 Jul;55:36.


Mutant SOD1 knockdown in all cell types ameliorates disease in G85R SOD1 mice with a limited additional effect over knockdown restricted to motor neurons.

Wang L, Grisotti G, Roos RP.

J Neurochem. 2010 Apr;113(1):166-74. doi: 10.1111/j.1471-4159.2010.06594.x. Epub 2010 Feb 2.


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.


Knocking down metabotropic glutamate receptor 1 improves survival and disease progression in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis.

Milanese M, Giribaldi F, Melone M, Bonifacino T, Musante I, Carminati E, Rossi PI, Vergani L, Voci A, Conti F, Puliti A, Bonanno G.

Neurobiol Dis. 2014 Apr;64:48-59. doi: 10.1016/j.nbd.2013.11.006. Epub 2013 Dec 19.


GLT1 overexpression in SOD1(G93A) mouse cervical spinal cord does not preserve diaphragm function or extend disease.

Li K, Hala TJ, Seetharam S, Poulsen DJ, Wright MC, Lepore AC.

Neurobiol Dis. 2015 Jun;78:12-23. doi: 10.1016/j.nbd.2015.03.010. Epub 2015 Mar 25.


Phenotype of transgenic mice carrying a very low copy number of the mutant human G93A superoxide dismutase-1 gene associated with amyotrophic lateral sclerosis.

Deitch JS, Alexander GM, Bensinger A, Yang S, Jiang JT, Heiman-Patterson TD.

PLoS One. 2014 Jun 19;9(6):e99879. doi: 10.1371/journal.pone.0099879. eCollection 2014.


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.


ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

Bruijn LI, Becher MW, Lee MK, Anderson KL, Jenkins NA, Copeland NG, Sisodia SS, Rothstein JD, Borchelt DR, Price DL, Cleveland DW.

Neuron. 1997 Feb;18(2):327-38.


Palmitoylation of superoxide dismutase 1 (SOD1) is increased for familial amyotrophic lateral sclerosis-linked SOD1 mutants.

Antinone SE, Ghadge GD, Lam TT, Wang L, Roos RP, Green WN.

J Biol Chem. 2013 Jul 26;288(30):21606-17. doi: 10.1074/jbc.M113.487231. Epub 2013 Jun 12.


Stabilization of mutant Cu/Zn superoxide dismutase (SOD1) protein by coexpressed wild SOD1 protein accelerates the disease progression in familial amyotrophic lateral sclerosis mice.

Fukada K, Nagano S, Satoh M, Tohyama C, Nakanishi T, Shimizu A, Yanagihara T, Sakoda S.

Eur J Neurosci. 2001 Dec;14(12):2032-6.


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.


Protein-bound crotonaldehyde accumulates in the spinal cord of superoxide dismutase-1 mutation-associated familial amyotrophic lateral sclerosis and its transgenic mouse model.

Shibata N, Kawaguchi M, Uchida K, Kakita A, Takahashi H, Nakano R, Fujimura H, Sakoda S, Ihara Y, Nobukuni K, Takehisa Y, Kuroda S, Kokubo Y, Kuzuhara S, Honma T, Mochizuki Y, Mizutani T, Yamada S, Toi S, Sasaki S, Iwata M, Hirano A, Yamamoto T, Kato Y, Sawada T, Kobayashi M.

Neuropathology. 2007 Feb;27(1):49-61.


Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways.

Ayers JI, Fromholt SE, O'Neal VM, Diamond JH, Borchelt DR.

Acta Neuropathol. 2016 Jan;131(1):103-14. doi: 10.1007/s00401-015-1514-0. Epub 2015 Dec 9.


The unfolded protein response in familial amyotrophic lateral sclerosis.

Wang L, Popko B, Roos RP.

Hum Mol Genet. 2011 Mar 1;20(5):1008-15. doi: 10.1093/hmg/ddq546. Epub 2010 Dec 15.


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