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Items: 1 to 20 of 115


Current hypotheses for the underlying biology of amyotrophic lateral sclerosis.

Rothstein JD.

Ann Neurol. 2009 Jan;65 Suppl 1:S3-9. doi: 10.1002/ana.21543. Review.


ALS: a disease of motor neurons and their nonneuronal neighbors.

Boillée S, Vande Velde C, Cleveland DW.

Neuron. 2006 Oct 5;52(1):39-59. Review.


Amyotrophic lateral sclerosis: from current developments in the laboratory to clinical implications.

Cozzolino M, Ferri A, Carrì MT.

Antioxid Redox Signal. 2008 Mar;10(3):405-43. doi: 10.1089/ars.2007.1760. Review.


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.


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.


Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis.

Parone PA, Da Cruz S, Han JS, McAlonis-Downes M, Vetto AP, Lee SK, Tseng E, Cleveland DW.

J Neurosci. 2013 Mar 13;33(11):4657-71. doi: 10.1523/JNEUROSCI.1119-12.2013.


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.


TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations.

Maekawa S, Leigh PN, King A, Jones E, Steele JC, Bodi I, Shaw CE, Hortobagyi T, Al-Sarraj S.

Neuropathology. 2009 Dec;29(6):672-83. doi: 10.1111/j.1440-1789.2009.01029.x. Epub 2009 Jun 3.


Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities.

Magrané J, Hervias I, Henning MS, Damiano M, Kawamata H, Manfredi G.

Hum Mol Genet. 2009 Dec 1;18(23):4552-64. doi: 10.1093/hmg/ddp421. Epub 2009 Sep 24.


Mitochondrial dysfunction and its role in motor neuron degeneration in ALS.

Manfredi G, Xu Z.

Mitochondrion. 2005 Apr;5(2):77-87. Review.


Endosomal accumulation of APP in wobbler motor neurons reflects impaired vesicle trafficking: implications for human motor neuron disease.

Palmisano R, Golfi P, Heimann P, Shaw C, Troakes C, Schmitt-John T, Bartsch JW.

BMC Neurosci. 2011 Mar 7;12:24. doi: 10.1186/1471-2202-12-24.


The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell death.

Muyderman H, Hutson PG, Matusica D, Rogers ML, Rush RA.

Neurochem Res. 2009 Oct;34(10):1847-56. doi: 10.1007/s11064-009-9974-z. Epub 2009 Apr 28. Review.


Human superoxide dismutase 1 overexpression in motor neurons of Caenorhabditis elegans causes axon guidance defect and neurodegeneration.

Li J, Li T, Zhang X, Tang Y, Yang J, Le W.

Neurobiol Aging. 2014 Apr;35(4):837-46. doi: 10.1016/j.neurobiolaging.2013.09.003. Epub 2013 Oct 11.


Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.

Mackenzie IR, Bigio EH, Ince PG, Geser F, Neumann M, Cairns NJ, Kwong LK, Forman MS, Ravits J, Stewart H, Eisen A, McClusky L, Kretzschmar HA, Monoranu CM, Highley JR, Kirby J, Siddique T, Shaw PJ, Lee VM, Trojanowski JQ.

Ann Neurol. 2007 May;61(5):427-34.


Mitochondrial dysfunction in amyotrophic lateral sclerosis.

Shi P, Gal J, Kwinter DM, Liu X, Zhu H.

Biochim Biophys Acta. 2010 Jan;1802(1):45-51. doi: 10.1016/j.bbadis.2009.08.012. Epub 2009 Aug 26. Review.


Rodent Models of Amyotrophic Lateral Sclerosis.

Philips T, Rothstein JD.

Curr Protoc Pharmacol. 2015 Jun 1;69:5.67.1-21. doi: 10.1002/0471141755.ph0567s69. Review.


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.

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