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Items: 1 to 20 of 371

1.

Inhibition of chaperone activity is a shared property of several Cu,Zn-superoxide dismutase mutants that cause amyotrophic lateral sclerosis.

Tummala H, Jung C, Tiwari A, Higgins CM, Hayward LJ, Xu Z.

J Biol Chem. 2005 May 6;280(18):17725-31. Epub 2005 Mar 7.

3.

Proteins that bind to misfolded mutant superoxide dismutase-1 in spinal cords from transgenic amyotrophic lateral sclerosis (ALS) model mice.

Zetterström P, Graffmo KS, Andersen PM, Brännström T, Marklund SL.

J Biol Chem. 2011 Jun 10;286(23):20130-6. doi: 10.1074/jbc.M111.218842. Epub 2011 Apr 14.

4.

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
5.

Differential effects of mutant SOD1 on protein structure of skeletal muscle and spinal cord of familial amyotrophic lateral sclerosis: role of chaperone network.

Wei R, Bhattacharya A, Hamilton RT, Jernigan AL, Chaudhuri AR.

Biochem Biophys Res Commun. 2013 Aug 16;438(1):218-23. doi: 10.1016/j.bbrc.2013.07.060. Epub 2013 Jul 23.

PMID:
23886956
6.
7.

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis.

Urushitani M, Sik A, Sakurai T, Nukina N, Takahashi R, Julien JP.

Nat Neurosci. 2006 Jan;9(1):108-18. Epub 2005 Dec 20.

PMID:
16369483
8.

Progressive impairment of CaV1.1 function in the skeletal muscle of mice expressing a mutant type 1 Cu/Zn superoxide dismutase (G93A) linked to amyotrophic lateral sclerosis.

Beqollari D, Romberg CF, Dobrowolny G, Martini M, Voss AA, Musarò A, Bannister RA.

Skelet Muscle. 2016 Jun 23;6:24. doi: 10.1186/s13395-016-0094-6. eCollection 2016.

9.

Transcriptional profiling in the lumbar spinal cord of a mouse model of amyotrophic lateral sclerosis: a role for wild-type superoxide dismutase 1 in sporadic disease?

D'Arrigo A, Colavito D, Peña-Altamira E, Fabris M, Dam M, Contestabile A, Leon A.

J Mol Neurosci. 2010 Jul;41(3):404-15. doi: 10.1007/s12031-010-9332-2. Epub 2010 Feb 23. Erratum in: J Mol Neurosci. 2010 Jul;41(3):416.

PMID:
20177826
11.

Different human copper-zinc superoxide dismutase mutants, SOD1G93A and SOD1H46R, exert distinct harmful effects on gross phenotype in mice.

Pan L, Yoshii Y, Otomo A, Ogawa H, Iwasaki Y, Shang HF, Hadano S.

PLoS One. 2012;7(3):e33409. doi: 10.1371/journal.pone.0033409. Epub 2012 Mar 16.

12.

In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space.

Igoudjil A, Magrané J, Fischer LR, Kim HJ, Hervias I, Dumont M, Cortez C, Glass JD, Starkov AA, Manfredi G.

J Neurosci. 2011 Nov 2;31(44):15826-37. doi: 10.1523/JNEUROSCI.1965-11.2011.

13.

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.

PMID:
11860498
14.

Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrophic lateral sclerosis.

Krishnan J, Vannuvel K, Andries M, Waelkens E, Robberecht W, Van Den Bosch L.

J Neurochem. 2008 Sep;106(5):2170-83. doi: 10.1111/j.1471-4159.2008.05545.x. Epub 2008 Jul 4.

15.

Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading.

Subramaniam JR, Lyons WE, Liu J, Bartnikas TB, Rothstein J, Price DL, Cleveland DW, Gitlin JD, Wong PC.

Nat Neurosci. 2002 Apr;5(4):301-7.

PMID:
11889469
16.

Delayed disease onset and extended survival in the SOD1G93A rat model of amyotrophic lateral sclerosis after suppression of mutant SOD1 in the motor cortex.

Thomsen GM, Gowing G, Latter J, Chen M, Vit JP, Staggenborg K, Avalos P, Alkaslasi M, Ferraiuolo L, Likhite S, Kaspar BK, Svendsen CN.

J Neurosci. 2014 Nov 19;34(47):15587-600. doi: 10.1523/JNEUROSCI.2037-14.2014.

17.
18.

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
19.

Amyotrophic lateral sclerosis-related mutant superoxide dismutase 1 aggregates inhibit 14-3-3-mediated cell survival by sequestration into the JUNQ compartment.

Park JH, Jang HR, Lee IY, Oh HK, Choi EJ, Rhim H, Kang S.

Hum Mol Genet. 2017 Sep 15;26(18):3615-3629. doi: 10.1093/hmg/ddx250.

PMID:
28666328
20.

Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis.

Novoselov SS, Mustill WJ, Gray AL, Dick JR, Kanuga N, Kalmar B, Greensmith L, Cheetham ME.

PLoS One. 2013 Aug 30;8(8):e73944. doi: 10.1371/journal.pone.0073944. eCollection 2013.

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