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Items: 1 to 20 of 313

1.

Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis.

Roberts BR, Lim NK, McAllum EJ, Donnelly PS, Hare DJ, Doble PA, Turner BJ, Price KA, Lim SC, Paterson BM, Hickey JL, Rhoads TW, Williams JR, Kanninen KM, Hung LW, Liddell JR, Grubman A, Monty JF, Llanos RM, Kramer DR, Mercer JF, Bush AI, Masters CL, Duce JA, Li QX, Beckman JS, Barnham KJ, White AR, Crouch PJ.

J Neurosci. 2014 Jun 4;34(23):8021-31. doi: 10.1523/JNEUROSCI.4196-13.2014.

2.

Zn II(atsm) is protective in amyotrophic lateral sclerosis model mice via a copper delivery mechanism.

McAllum EJ, Roberts BR, Hickey JL, Dang TN, Grubman A, Donnelly PS, Liddell JR, White AR, Crouch PJ.

Neurobiol Dis. 2015 Sep;81:20-4. doi: 10.1016/j.nbd.2015.02.023. Epub 2015 Mar 10.

3.

Therapeutic effects of CuII(atsm) in the SOD1-G37R mouse model of amyotrophic lateral sclerosis.

McAllum EJ, Lim NK, Hickey JL, Paterson BM, Donnelly PS, Li QX, Liddell JR, Barnham KJ, White AR, Crouch PJ.

Amyotroph Lateral Scler Frontotemporal Degener. 2013 Dec;14(7-8):586-90. doi: 10.3109/21678421.2013.824000. Epub 2013 Aug 19.

PMID:
23952668
4.

Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model.

Soon CP, Donnelly PS, Turner BJ, Hung LW, Crouch PJ, Sherratt NA, Tan JL, Lim NK, Lam L, Bica L, Lim S, Hickey JL, Morizzi J, Powell A, Finkelstein DI, Culvenor JG, Masters CL, Duce J, White AR, Barnham KJ, Li QX.

J Biol Chem. 2011 Dec 23;286(51):44035-44. doi: 10.1074/jbc.M111.274407. Epub 2011 Oct 27.

5.

CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord.

Hilton JB, Mercer SW, Lim NK, Faux NG, Buncic G, Beckman JS, Roberts BR, Donnelly PS, White AR, Crouch PJ.

Sci Rep. 2017 Feb 13;7:42292. doi: 10.1038/srep42292.

6.

Heat shock factor 1 over-expression protects against exposure of hydrophobic residues on mutant SOD1 and early mortality in a mouse model of amyotrophic lateral sclerosis.

Lin PY, Simon SM, Koh WK, Folorunso O, Umbaugh CS, Pierce A.

Mol Neurodegener. 2013 Nov 21;8:43. doi: 10.1186/1750-1326-8-43.

7.

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
9.

Genetically decreased spinal cord copper concentration prolongs life in a transgenic mouse model of amyotrophic lateral sclerosis.

Kiaei M, Bush AI, Morrison BM, Morrison JH, Cherny RA, Volitakis I, Beal MF, Gordon JW.

J Neurosci. 2004 Sep 8;24(36):7945-50.

10.

A cysteine residue affects the conformational state and neuronal toxicity of mutant SOD1 in mice: relevance to the pathogenesis of ALS.

Nagano S, Takahashi Y, Yamamoto K, Masutani H, Fujiwara N, Urushitani M, Araki T.

Hum Mol Genet. 2015 Jun 15;24(12):3427-39. doi: 10.1093/hmg/ddv093. Epub 2015 Mar 11.

PMID:
25762155
12.

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.

13.

Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice.

Lelie HL, Liba A, Bourassa MW, Chattopadhyay M, Chan PK, Gralla EB, Miller LM, Borchelt DR, Valentine JS, Whitelegge JP.

J Biol Chem. 2011 Jan 28;286(4):2795-806. doi: 10.1074/jbc.M110.186999. Epub 2010 Nov 10.

14.

Neuregulin 1 confers neuroprotection in SOD1-linked amyotrophic lateral sclerosis mice via restoration of C-boutons of spinal motor neurons.

Lasiene J, Komine O, Fujimori-Tonou N, Powers B, Endo F, Watanabe S, Shijie J, Ravits J, Horner P, Misawa H, Yamanaka K.

Acta Neuropathol Commun. 2016 Feb 18;4:15. doi: 10.1186/s40478-016-0286-7.

15.

Dysregulation of intracellular copper homeostasis is common to transgenic mice expressing human mutant superoxide dismutase-1s regardless of their copper-binding abilities.

Tokuda E, Okawa E, Watanabe S, Ono S, Marklund SL.

Neurobiol Dis. 2013 Jun;54:308-19. doi: 10.1016/j.nbd.2013.01.001. Epub 2013 Jan 13.

PMID:
23321002
16.

Neuroprotective effects of the Sigma-1 receptor (S1R) agonist PRE-084, in a mouse model of motor neuron disease not linked to SOD1 mutation.

Peviani M, Salvaneschi E, Bontempi L, Petese A, Manzo A, Rossi D, Salmona M, Collina S, Bigini P, Curti D.

Neurobiol Dis. 2014 Feb;62:218-32. doi: 10.1016/j.nbd.2013.10.010. Epub 2013 Oct 16.

PMID:
24141020
17.

Increased affinity for copper mediated by cysteine 111 in forms of mutant superoxide dismutase 1 linked to amyotrophic lateral sclerosis.

Watanabe S, Nagano S, Duce J, Kiaei M, Li QX, Tucker SM, Tiwari A, Brown RH Jr, Beal MF, Hayward LJ, Culotta VC, Yoshihara S, Sakoda S, Bush AI.

Free Radic Biol Med. 2007 May 15;42(10):1534-42. Epub 2007 Feb 15.

PMID:
17448900
18.

Targeting of monomer/misfolded SOD1 as a therapeutic strategy for amyotrophic lateral sclerosis.

Liu HN, Tjostheim S, Dasilva K, Taylor D, Zhao B, Rakhit R, Brown M, Chakrabartty A, McLaurin J, Robertson J.

J Neurosci. 2012 Jun 27;32(26):8791-9. doi: 10.1523/JNEUROSCI.5053-11.2012.

19.

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
20.

Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.

Clement AM, Nguyen MD, Roberts EA, Garcia ML, Boillée S, Rule M, McMahon AP, Doucette W, Siwek D, Ferrante RJ, Brown RH Jr, Julien JP, Goldstein LS, Cleveland DW.

Science. 2003 Oct 3;302(5642):113-7. Erratum in: Science. 2003 Oct 24;302(5645):568.

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