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Items: 1 to 20 of 111

1.

Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm.

Song Y, Nagy M, Ni W, Tyagi NK, Fenton WA, López-Giráldez F, Overton JD, Horwich AL, Brady ST.

Proc Natl Acad Sci U S A. 2013 Apr 2;110(14):5428-33. doi: 10.1073/pnas.1303279110. Epub 2013 Mar 18.

2.

Inhibition of fast axonal transport by pathogenic SOD1 involves activation of p38 MAP kinase.

Morfini GA, Bosco DA, Brown H, Gatto R, Kaminska A, Song Y, Molla L, Baker L, Marangoni MN, Berth S, Tavassoli E, Bagnato C, Tiwari A, Hayward LJ, Pigino GF, Watterson DM, Huang CF, Banker G, Brown RH Jr, Brady ST.

PLoS One. 2013 Jun 12;8(6):e65235. doi: 10.1371/journal.pone.0065235. Print 2013.

3.

Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110.

Nagy M, Fenton WA, Li D, Furtak K, Horwich AL.

Proc Natl Acad Sci U S A. 2016 May 10;113(19):5424-8. doi: 10.1073/pnas.1604885113. Epub 2016 Apr 25.

4.

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.

5.

Inhibition of p38 mitogen activated protein kinase activation and mutant SOD1(G93A)-induced motor neuron death.

Dewil M, dela Cruz VF, Van Den Bosch L, Robberecht W.

Neurobiol Dis. 2007 May;26(2):332-41. Epub 2007 Feb 6.

PMID:
17346981
6.

Absence of lipofuscin in motor neurons of SOD1-linked ALS mice.

Bandyopadhyay U, Nagy M, Fenton WA, Horwich AL.

Proc Natl Acad Sci U S A. 2014 Jul 29;111(30):11055-60. doi: 10.1073/pnas.1409314111. Epub 2014 Jul 14.

7.

Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transport.

Shi P, Ström AL, Gal J, Zhu H.

Biochim Biophys Acta. 2010 Sep;1802(9):707-16. doi: 10.1016/j.bbadis.2010.05.008. Epub 2010 May 25.

8.

Interaction between familial amyotrophic lateral sclerosis (ALS)-linked SOD1 mutants and the dynein complex.

Zhang F, Ström AL, Fukada K, Lee S, Hayward LJ, Zhu H.

J Biol Chem. 2007 Jun 1;282(22):16691-9. Epub 2007 Apr 2.

9.

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS).

Crippa V, Sau D, Rusmini P, Boncoraglio A, Onesto E, Bolzoni E, Galbiati M, Fontana E, Marino M, Carra S, Bendotti C, De Biasi S, Poletti A.

Hum Mol Genet. 2010 Sep 1;19(17):3440-56. doi: 10.1093/hmg/ddq257. Epub 2010 Jun 22.

PMID:
20570967
10.

Heat-shock protein 105 interacts with and suppresses aggregation of mutant Cu/Zn superoxide dismutase: clues to a possible strategy for treating ALS.

Yamashita H, Kawamata J, Okawa K, Kanki R, Nakamizo T, Hatayama T, Yamanaka K, Takahashi R, Shimohama S.

J Neurochem. 2007 Sep;102(5):1497-505. Epub 2007 Mar 30.

11.

Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS.

Wang J, Farr GW, Zeiss CJ, Rodriguez-Gil DJ, Wilson JH, Furtak K, Rutkowski DT, Kaufman RJ, Ruse CI, Yates JR 3rd, Perrin S, Feany MB, Horwich AL.

Proc Natl Acad Sci U S A. 2009 Feb 3;106(5):1392-7. doi: 10.1073/pnas.0813045106. Epub 2009 Jan 26.

12.

Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice.

Borchelt DR, Wong PC, Becher MW, Pardo CA, Lee MK, Xu ZS, Thinakaran G, Jenkins NA, Copeland NG, Sisodia SS, Cleveland DW, Price DL, Hoffman PN.

Neurobiol Dis. 1998 Jul;5(1):27-35.

PMID:
9702785
13.

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.

14.

Hydrogen-deuterium exchange in vivo to measure turnover of an ALS-associated mutant SOD1 protein in spinal cord of mice.

Farr GW, Ying Z, Fenton WA, Horwich AL.

Protein Sci. 2011 Oct;20(10):1692-6. doi: 10.1002/pro.700. Epub 2011 Aug 18.

15.

Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content.

De Vos KJ, Chapman AL, Tennant ME, Manser C, Tudor EL, Lau KF, Brownlees J, Ackerley S, Shaw PJ, McLoughlin DM, Shaw CE, Leigh PN, Miller CC, Grierson AJ.

Hum Mol Genet. 2007 Nov 15;16(22):2720-8. Epub 2007 Aug 28.

16.

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
17.

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.

19.

Activation of the p38MAPK cascade is associated with upregulation of TNF alpha receptors in the spinal motor neurons of mouse models of familial ALS.

Veglianese P, Lo Coco D, Bao Cutrona M, Magnoni R, Pennacchini D, Pozzi B, Gowing G, Julien JP, Tortarolo M, Bendotti C.

Mol Cell Neurosci. 2006 Feb;31(2):218-31. Epub 2005 Oct 10.

PMID:
16219474
20.

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: neuroprotection by SIRT3 and PGC-1α.

Song W, Song Y, Kincaid B, Bossy B, Bossy-Wetzel E.

Neurobiol Dis. 2013 Mar;51:72-81. doi: 10.1016/j.nbd.2012.07.004. Epub 2012 Jul 20.

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