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Items: 1 to 20 of 138

1.

Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons.

Wainger BJ, Kiskinis E, Mellin C, Wiskow O, Han SS, Sandoe J, Perez NP, Williams LA, Lee S, Boulting G, Berry JD, Brown RH Jr, Cudkowicz ME, Bean BP, Eggan K, Woolf CJ.

Cell Rep. 2014 Apr 10;7(1):1-11. doi: 10.1016/j.celrep.2014.03.019. Epub 2014 Apr 3.

2.

Human embryonic stem cell-derived motor neurons expressing SOD1 mutants exhibit typical signs of motor neuron degeneration linked to ALS.

Karumbayaram S, Kelly TK, Paucar AA, Roe AJ, Umbach JA, Charles A, Goldman SA, Kornblum HI, Wiedau-Pazos M.

Dis Model Mech. 2009 Mar-Apr;2(3-4):189-95. doi: 10.1242/dmm.002113. Epub 2009 Feb 23.

3.

4-Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient-Derived Induced Pluripotent Stem Cells.

Naujock M, Stanslowsky N, Bufler S, Naumann M, Reinhardt P, Sterneckert J, Kefalakes E, Kassebaum C, Bursch F, Lojewski X, Storch A, Frickenhaus M, Boeckers TM, Putz S, Demestre M, Liebau S, Klingenstein M, Ludolph AC, Dengler R, Kim KS, Hermann A, Wegner F, Petri S.

Stem Cells. 2016 Jun;34(6):1563-75. doi: 10.1002/stem.2354. Epub 2016 Mar 28.

4.

Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1.

Kiskinis E, Sandoe J, Williams LA, Boulting GL, Moccia R, Wainger BJ, Han S, Peng T, Thams S, Mikkilineni S, Mellin C, Merkle FT, Davis-Dusenbery BN, Ziller M, Oakley D, Ichida J, Di Costanzo S, Atwater N, Maeder ML, Goodwin MJ, Nemesh J, Handsaker RE, Paull D, Noggle S, McCarroll SA, Joung JK, Woolf CJ, Brown RH, Eggan K.

Cell Stem Cell. 2014 Jun 5;14(6):781-95. doi: 10.1016/j.stem.2014.03.004. Epub 2014 Apr 3.

5.

Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis.

Turner BJ, Atkin JD, Farg MA, Zang DW, Rembach A, Lopes EC, Patch JD, Hill AF, Cheema SS.

J Neurosci. 2005 Jan 5;25(1):108-17.

6.

A drosophila model for amyotrophic lateral sclerosis reveals motor neuron damage by human SOD1.

Watson MR, Lagow RD, Xu K, Zhang B, Bonini NM.

J Biol Chem. 2008 Sep 5;283(36):24972-81. doi: 10.1074/jbc.M804817200. Epub 2008 Jul 2.

7.

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.

8.

Transcriptomic indices of fast and slow disease progression in two mouse models of amyotrophic lateral sclerosis.

Nardo G, Iennaco R, Fusi N, Heath PR, Marino M, Trolese MC, Ferraiuolo L, Lawrence N, Shaw PJ, Bendotti C.

Brain. 2013 Nov;136(Pt 11):3305-32. doi: 10.1093/brain/awt250. Epub 2013 Sep 24.

PMID:
24065725
9.

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.

10.

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.

11.

Modeling amyotrophic lateral sclerosis in pure human iPSc-derived motor neurons isolated by a novel FACS double selection technique.

Toli D, Buttigieg D, Blanchard S, Lemonnier T, Lamotte d'Incamps B, Bellouze S, Baillat G, Bohl D, Haase G.

Neurobiol Dis. 2015 Oct;82:269-280. doi: 10.1016/j.nbd.2015.06.011. Epub 2015 Jun 21.

PMID:
26107889
12.

Motor neurons derived from ALS-related mouse iPS cells recapitulate pathological features of ALS.

Park JH, Park HS, Hong S, Kang S.

Exp Mol Med. 2016 Dec 9;48(12):e276. doi: 10.1038/emm.2016.113.

13.

Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons.

Lobsiger CS, Boillée S, Cleveland DW.

Proc Natl Acad Sci U S A. 2007 May 1;104(18):7319-26. Epub 2007 Apr 26.

14.

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.

15.

Non-cell-autonomous effect of human SOD1 G37R astrocytes on motor neurons derived from human embryonic stem cells.

Marchetto MC, Muotri AR, Mu Y, Smith AM, Cezar GG, Gage FH.

Cell Stem Cell. 2008 Dec 4;3(6):649-57. doi: 10.1016/j.stem.2008.10.001.

16.

A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells.

Burkhardt MF, Martinez FJ, Wright S, Ramos C, Volfson D, Mason M, Garnes J, Dang V, Lievers J, Shoukat-Mumtaz U, Martinez R, Gai H, Blake R, Vaisberg E, Grskovic M, Johnson C, Irion S, Bright J, Cooper B, Nguyen L, Griswold-Prenner I, Javaherian A.

Mol Cell Neurosci. 2013 Sep;56:355-64. doi: 10.1016/j.mcn.2013.07.007. Epub 2013 Jul 25.

18.

Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis.

Pieri M, Albo F, Gaetti C, Spalloni A, Bengtson CP, Longone P, Cavalcanti S, Zona C.

Neurosci Lett. 2003 Nov 20;351(3):153-6.

PMID:
14623129
19.

Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice.

Yamanaka K, Boillee S, Roberts EA, Garcia ML, McAlonis-Downes M, Mikse OR, Cleveland DW, Goldstein LS.

Proc Natl Acad Sci U S A. 2008 May 27;105(21):7594-9. doi: 10.1073/pnas.0802556105. Epub 2008 May 20.

20.

Amyotrophic lateral sclerosis models derived from human embryonic stem cells with different superoxide dismutase 1 mutations exhibit differential drug responses.

Isobe T, Tooi N, Nakatsuji N, Aiba K.

Stem Cell Res. 2015 Nov;15(3):459-468. doi: 10.1016/j.scr.2015.09.006. Epub 2015 Sep 24.

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