SOD1 Rat Publications

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Thomsen GM, et al.
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Thomsen GM, Vit JP, Lamb A, Gowing G, Shelest O, Alkaslasi M, Ley EJ, Svendsen CN
(2015) Acute Traumatic Brain Injury Does Not Exacerbate Amyotrophic Lateral Sclerosis in the SOD1 (G93A) Rat Model eNeuro 2(3)
Neuroscience
McCampbell A, et al.
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McCampbell A, Cole T, Wegener AJ, Tomassy GS, Setnicka A, Farley BJ, Schoch KM, Hoye ML, Shabsovich M, Sun L, Luo Y, Zhang M, Comfort N, Wang B, Amacker J, Thankamony S, Salzman DW, Cudkowicz M, Graham DL, Bennett CF, Kordasiewicz HB, Swayze EE, Miller TM
(2018) Antisense oligonucleotides extend survival and reverse decrement in muscle response in ALS models. J Clin Invest 128(8):3558-3567
shRNA-mediatred gene knock down
Neuroscience
Smith RA, et al.
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Smith RA, Miller TM, Yamanaka K, Monia BP, Condon TP, Hung G, Lobsiger CS, Ward CM, McAlonis-Downes M, Wei H, Wancewicz EV, Bennett CF, Cleveland DW
(2006) Antisense oligonucleotide therapy for neurodegenerative disease. J Clin Invest 116(8):2290-6
Neuroscience
Thonhoff JR, et al.
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Thonhoff JR, Jordan PM, Karam JR, Bassett BL, Wu P
(2007) Identification of early disease progression in an ALS rat model. Neurosci Lett 415(3):264-8
Neuroscience
Lobsiger CS, et al.
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Lobsiger CS, Boillée S, Cleveland DW
(2007) Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A 104(18):7319-26
Neuroscience
Pehar M, et al.
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Pehar M, Vargas MR, Robinson KM, Cassina P, Díaz-Amarilla PJ, Hagen TM, Radi R, Barbeito L, Beckman JS
(2007) Mitochondrial superoxide production and nuclear factor erythroid 2-related factor 2 activation in p75 neurotrophin receptor-induced motor neuron apoptosis. J Neurosci 27(29):7777-85
Neuroscience
Suzuki M, et al.
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Suzuki M, McHugh J, Tork C, Shelley B, Klein SM, Aebischer P, Svendsen CN
(2007) GDNF secreting human neural progenitor cells protect dying motor neurons, but not their projection to muscle, in a rat model of familial ALS. PLoS One 2(8):e689
Neuroscience
Yin HZ, et al.
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Yin HZ, Tang DT, Weiss JH
(2007) Intrathecal infusion of a Ca(2+)-permeable AMPA channel blocker slows loss of both motor neurons and of the astrocyte glutamate transporter, GLT-1 in a mutant SOD1 rat model of ALS. Exp Neurol 207(2):177-85
Neuroscience
Van Damme P, et al.
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Van Damme P, Bogaert E, Dewil M, Hersmus N, Kiraly D, Scheveneels W, Bockx I, Braeken D, Verpoorten N, Verhoeven K, Timmerman V, Herijgers P, Callewaert G, Carmeliet P, Van Den Bosch L, Robberecht W
(2007) Astrocytes regulate GluR2 expression in motor neurons and their vulnerability to excitotoxicity. Proc Natl Acad Sci U S A 104(37):14825-30
Neuroscience
Cassina P, et al.
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Cassina P, Cassina A, Pehar M, Castellanos R, Gandelman M, de León A, Robinson KM, Mason RP, Beckman JS, Barbeito L, Radi R
(2008) Mitochondrial dysfunction in SOD1G93A-bearing astrocytes promotes motor neuron degeneration: prevention by mitochondrial-targeted antioxidants. J Neurosci 28(16):4115-22
Neuroscience
Vargas MR, et al.
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Vargas MR, Pehar M, Díaz-Amarilla PJ, Beckman JS, Barbeito L
(2008) Transcriptional profile of primary astrocytes expressing ALS-linked mutant SOD1. J Neurosci Res 86(16):3515-25
Neuroscience
Suzuki M, et al.
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Suzuki M, McHugh J, Tork C, Shelley B, Hayes A, Bellantuono I, Aebischer P, Svendsen CN
(2008) Direct muscle delivery of GDNF with human mesenchymal stem cells improves motor neuron survival and function in a rat model of familial ALS. Mol Ther 16(12):2002-10
Neuroscience
Yang Y, et al.
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Yang Y, Gozen O, Watkins A, Lorenzini I, Lepore A, Gao Y, Vidensky S, Brennan J, Poulsen D, Won Park J, Li Jeon N, Robinson MB, Rothstein JD
(2009) Presynaptic regulation of astroglial excitatory neurotransmitter transporter GLT1. Neuron 61(6):880-94
Neuroscience
Sotelo-Silveira JR, et al.
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Sotelo-Silveira JR, Lepanto P, Elizondo V, Horjales S, Palacios F, Martinez-Palma L, Marin M, Beckman JS, Barbeito L
(2009) Axonal mitochondrial clusters containing mutant SOD1 in transgenic models of ALS. Antioxid Redox Signal 11(7):1535-45
Neuroscience
Franz CK, et al.
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Franz CK, Quach ET, Krudy CA, Federici T, Kliem MA, Snyder BR, Raore B, Boulis NM
(2009) A conditioning lesion provides selective protection in a rat model of Amyotrophic Lateral Sclerosis. PLoS One 4(10):e7357
Neuroscience
Suzuki M, et al.
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Suzuki M, Klein S, Wetzel EA, Meyer M, McHugh J, Tork C, Hayes A, Svendsen CN
(2010) Acute glial activation by stab injuries does not lead to overt damage or motor neuron degeneration in the G93A mutant SOD1 rat model of amyotrophic lateral sclerosis. Exp Neurol 221(2):346-52
Neuroscience
Choi JK, et al.
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Choi JK, Dedeoglu A, Jenkins BG
(2010) Longitudinal monitoring of motor neuron circuitry in FALS rats using in-vivo phMRI. Neuroreport 21(3):157-62
Neuroscience
Smittkamp SE, et al.
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Smittkamp SE, Spalding HN, Brown JW, Gupte AA, Chen J, Nishimune H, Geiger PC, Stanford JA
(2010) Measures of bulbar and spinal motor function, muscle innervation, and mitochondrial function in ALS rats. Behav Brain Res 211(1):48-57
Neuroscience
Hedlund E, et al.
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Hedlund E, Karlsson M, Osborn T, Ludwig W, Isacson O
(2010) Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protection. Brain 133(Pt 8):2313-30
Neuroscience
Xu L, et al.
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Xu L, Shen P, Hazel T, Johe K, Koliatsos VE
(2011) Dual transplantation of human neural stem cells into cervical and lumbar cord ameliorates motor neuron disease in SOD1 transgenic rats. Neurosci Lett 494(3):222-6
Neuroscience
Rhoads TW, et al.
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Rhoads TW, Lopez NI, Zollinger DR, Morré JT, Arbogast BL, Maier CS, DeNoyer L, Beckman JS
(2011) Measuring copper and zinc superoxide dismutase from spinal cord tissue using electrospray mass spectrometry. Anal Biochem 415(1):52-8
Neuroscience
Berger JV, et al.
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Berger JV, Deumens R, Goursaud S, Schäfer S, Lavand'homme P, Joosten EA, Hermans E
(2011) Enhanced neuroinflammation and pain hypersensitivity after peripheral nerve injury in rats expressing mutated superoxide dismutase 1. J Neuroinflammation 8:33
Neuroscience
Díaz-Amarilla P, et al.
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Díaz-Amarilla P, Olivera-Bravo S, Trias E, Cragnolini A, Martínez-Palma L, Cassina P, Beckman J, Barbeito L
(2011) Phenotypically aberrant astrocytes that promote motoneuron damage in a model of inherited amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 108(44):18126-31
Neuroscience
Magrané J, et al.
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Magrané J, Sahawneh MA, Przedborski S, Estévez ÁG, Manfredi G
(2012) Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons. J Neurosci 32(1):229-42
Neuroscience
Yin HZ, et al.
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Yin HZ, Weiss JH
(2012) Marked synergism between mutant SOD1 and glutamate transport inhibition in the induction of motor neuronal degeneration in spinal cord slice cultures. Brain Res 1448:153-62
Neuroscience
Hefferan MP, et al.
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Hefferan MP, Galik J, Kakinohana O, Sekerkova G, Santucci C, Marsala S, Navarro R, Hruska-Plochan M, Johe K, Feldman E, Cleveland DW, Marsala M
(2012) Human neural stem cell replacement therapy for amyotrophic lateral sclerosis by spinal transplantation. PLoS One 7(8):e42614
Neuroscience
Li R, et al.
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Li R, Strykowski R, Meyer M, Mulcrone P, Krakora D, Suzuki M
(2012) Male-specific differences in proliferation, neurogenesis, and sensitivity to oxidative stress in neural progenitor cells derived from a rat model of ALS. PLoS One 7(11):e48581
Neuroscience
Winer L, et al.
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Winer L, Srinivasan D, Chun S, Lacomis D, Jaffa M, Fagan A, Holtzman DM, Wancewicz E, Bennett CF, Bowser R, Cudkowicz M, Miller TM
(2013) SOD1 in cerebral spinal fluid as a pharmacodynamic marker for antisense oligonucleotide therapy. JAMA Neurol 70(2):201-7
Neuroscience
Linares E, et al.
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Linares E, Seixas LV, dos Prazeres JN, Ladd FV, Ladd AA, Coppi AA, Augusto O
(2013) Tempol moderately extends survival in a hSOD1(G93A) ALS rat model by inhibiting neuronal cell loss, oxidative damage and levels of non-native hSOD1(G93A) forms. PLoS One 8(2):e55868
Neuroscience
Thonhoff JR, et al.
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Thonhoff JR, Gao J, Dunn TJ, Ojeda L, Wu P
(2012) Mutant SOD1 microglia-generated nitroxidative stress promotes toxicity to human fetal neural stem cell-derived motor neurons through direct damage and noxious interactions with astrocytes. Am J Stem Cells 1(1):2-21
Neuroscience
Krakora D, et al.
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Krakora D, Mulcrone P, Meyer M, Lewis C, Bernau K, Gowing G, Zimprich C, Aebischer P, Svendsen CN, Suzuki M
(2013) Synergistic effects of GDNF and VEGF on lifespan and disease progression in a familial ALS rat model. Mol Ther 21(8):1602-10
Neuroscience
Trias E, et al.
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Trias E, Díaz-Amarilla P, Olivera-Bravo S, Isasi E, Drechsel DA, Lopez N, Bradford CS, Ireton KE, Beckman JS, Barbeito L
(2013) Phenotypic transition of microglia into astrocyte-like cells associated with disease onset in a model of inherited ALS. Front Cell Neurosci 7:274
Neuroscience
Nichols NL, et al.
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Nichols NL, Johnson RA, Satriotomo I, Mitchell GS
(2014) Neither serotonin nor adenosine-dependent mechanisms preserve ventilatory capacity in ALS rats. Respir Physiol Neurobiol 197:19-28
Neuroscience
Dumont AO, et al.
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Dumont AO, Goursaud S, Desmet N, Hermans E
(2014) Differential regulation of glutamate transporter subtypes by pro-inflammatory cytokine TNF-α in cortical astrocytes from a rat model of amyotrophic lateral sclerosis. PLoS One 9(5):e97649
Neuroscience
Popescu IR, et al.
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Popescu IR, Nicaise C, Liu S, Bisch G, Knippenberg S, Daubie V, Bohl D, Pochet R
(2013) Neural progenitors derived from human induced pluripotent stem cells survive and differentiate upon transplantation into a rat model of amyotrophic lateral sclerosis. Stem Cells Transl Med 2(3):167-74
Neuroscience
Yin HZ, et al.
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Yin HZ, Yu S, Hsu CI, Liu J, Acab A, Wu R, Tao A, Chiang BJ, Weiss JH
(2014) Intrathecal infusion of BMAA induces selective motor neuron damage and astrogliosis in the ventral horn of the spinal cord. Exp Neurol 261:1-9
Neuroscience
Israelson A, et al.
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Israelson A, Ditsworth D, Sun S, Song S, Liang J, Hruska-Plochan M, McAlonis-Downes M, Abu-Hamad S, Zoltsman G, Shani T, Maldonado M, Bui A, Navarro M, Zhou H, Marsala M, Kaspar BK, Da Cruz S, Cleveland DW
(2015) Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1. Neuron 86(1):218-32
Neuroscience
Van Dyke JM, et al.
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Van Dyke JM, Smit-Oistad IM, Macrander C, Krakora D, Meyer MG, Suzuki M
(2016) Macrophage-mediated inflammation and glial response in the skeletal muscle of a rat model of familial amyotrophic lateral sclerosis (ALS). Exp Neurol 277:275-282
Neuroscience
Diaz-Amarilla P, et al.
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Diaz-Amarilla P, Miquel E, Trostchansky A, Trias E, Ferreira AM, Freeman BA, Cassina P, Barbeito L, Vargas MR, Rubbo H
(2016) Electrophilic nitro-fatty acids prevent astrocyte-mediated toxicity to motor neurons in a cell model of familial amyotrophic lateral sclerosis via nuclear factor erythroid 2-related factor activation. Free Radic Biol Med 95:112-20
Neuroscience
Pickles S, et al.
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Pickles S, Semmler S, Broom HR, Destroismaisons L, Legroux L, Arbour N, Meiering E, Cashman NR, Vande Velde C
(2016) ALS-linked misfolded SOD1 species have divergent impacts on mitochondria. Acta Neuropathol Commun 4(1):43
Neuroscience
Trias E, et al.
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Trias E, Ibarburu S, Barreto-Núñez R, Varela V, Moura IC, Dubreuil P, Hermine O, Beckman JS, Barbeito L
(2017) Evidence for mast cells contributing to neuromuscular pathology in an inherited model of ALS. JCI Insight 2(20)
Neuroscience
Seven YB, et al.
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Seven YB, Nichols NL, Kelly MN, Hobson OR, Satriotomo I, Mitchell GS
(2018) Compensatory plasticity in diaphragm and intercostal muscle utilization in a rat model of ALS. Exp Neurol 299(Pt A):148-156
Neuroscience
Dantas LS, et al.
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Dantas LS, Chaves-Filho AB, Coelho FR, Genaro-Mattos TC, Tallman KA, Porter NA, Augusto O, Miyamoto S
(2018) Cholesterol secosterol aldehyde adduction and aggregation of Cu,Zn-superoxide dismutase: Potential implications in ALS. Redox Biol 19:105-115
Neuroscience
Jeffrey J, et al.
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Jeffrey J, D'Cunha H, Suzuki M
(2018) Blood Level of Glial Fibrillary Acidic Protein (GFAP) Does not Correlate With Disease Progression in a Rat Model of Familial ALS (SOD1 Transgenic). Front Neurol 9:954
Neuroscience
Martínez-Palma L, et al.
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Martínez-Palma L, Miquel E, Lagos-Rodríguez V, Barbeito L, Cassina A, Cassina P
(2019) Mitochondrial Modulation by Dichloroacetate Reduces Toxicity of Aberrant Glial Cells and Gliosis in the SOD1G93A Rat Model of Amyotrophic Lateral Sclerosis. Neurotherapeutics 16(1):203-215
Neuroscience
Trias E, et al.
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Trias E, Beilby PR, Kovacs M, Ibarburu S, Varela V, Barreto-Núñez R, Bradford SC, Beckman JS, Barbeito L
(2019) Emergence of Microglia Bearing Senescence Markers During Paralysis Progression in a Rat Model of Inherited ALS. Front Aging Neurosci 11:42
Neuroscience
Gertsman I, et al.
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Gertsman I, Wuu J, McAlonis-Downes M, Ghassemian M, Ling K, Rigo F, Bennett F, Benatar M, Miller TM, Da Cruz S
(2019) An endogenous peptide marker differentiates SOD1 stability and facilitates pharmacodynamic monitoring in SOD1 amyotrophic lateral sclerosis. JCI Insight 4(10)
Neuroscience
Andjus P, et al.
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Andjus P, Stamenković S, Dučić T
(2019) Synchrotron radiation-based FTIR spectro-microscopy of the brainstem of the hSOD1 G93A rat model of amyotrophic lateral sclerosis. Eur Biophys J 48(5):475-484
Neuroscience
Kovacs M, et al.
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Kovacs M, Trias E, Varela V, Ibarburu S, Beckman JS, Moura IC, Hermine O, King PH, Si Y, Kwon Y, Barbeito L
(2019) CD34 Identifies a Subset of Proliferating Microglial Cells Associated with Degenerating Motor Neurons in ALS. Int J Mol Sci 20(16)
Neuroscience
Harrigan ME, et al.
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Harrigan ME, Filous AR, Tosolini AP, Morris R, Schwab JM, Arnold WD
(2019) Assessing Rat Forelimb and Hindlimb Motor Unit Connectivity as Objective and Robust Biomarkers of Spinal Motor Neuron Function. Sci Rep 9(1):16699
Neuroscience
Semmler S, et al.
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Semmler S, Gagné M, Garg P, Pickles SR, Baudouin C, Hamon-Keromen E, Destroismaisons L, Khalfallah Y, Chaineau M, Caron E, Bayne AN, Trempe JF, Cashman NR, Star AT, Haqqani AS, Durcan TM, Meiering EM, Robertson J, Grandvaux N, Plotkin SS, McBride HM, Vande Velde C
(2020) TNF receptor-associated factor 6 interacts with ALS-linked misfolded superoxide dismutase 1 and promotes aggregation. J Biol Chem 295(12):3808-3825
Neuroscience
Pickles S, et al.
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Pickles S, Destroismaisons L, Peyrard SL, Cadot S, Rouleau GA, Brown RH, Julien JP, Arbour N, Vande Velde C
(2013) Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. Hum Mol Genet 22(19):3947-59
Neuroscience
Ibarburu S, et al.
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Ibarburu S, Kovacs M, Varela V, Rodríguez-Duarte J, Ingold M, Invernizzi P, Porcal W, Arévalo AP, Perelmuter K, Bollati-Fogolín M, Escande C, López GV, King P, Si Y, Kwon Y, Batthyány C, Barbeito L, Trias E
(2021) A Nitroalkene Benzoic Acid Derivative Targets Reactive Microglia and Prolongs Survival in an Inherited Model of ALS via NF-κB Inhibition. Neurotherapeutics 18(1):309-325
Neuroscience
Lynch EM, et al.
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Lynch EM, Robertson S, FitzGibbons C, Reilly M, Switalski C, Eckardt A, Tey SR, Hayakawa K, Suzuki M
(2021) Transcriptome analysis using patient iPSC-derived skeletal myocytes: Bet1L as a new molecule possibly linked to neuromuscular junction degeneration in ALS. Exp Neurol 345:113815
Neuroscience
Klein SM, et al.
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Klein SM, Behrstock S, McHugh J, Hoffmann K, Wallace K, Suzuki M, Aebischer P, Svendsen CN
(2005) GDNF delivery using human neural progenitor cells in a rat model of ALS. Hum Gene Ther 16(4):509-21
Neuroscience, Gene/Cell therapy
Turner BJ, et al.
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Turner BJ, Atkin JD, Farg MA, Zang DW, Rembach A, Lopes EC, Patch JD, Hill AF, Cheema SS
(2005) Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis. J Neurosci 25(1):108-17
Neuroscience
Lepore AC, et al.
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Lepore AC, Tolmie C, O'Donnell J, Wright MC, Dejea C, Rauck B, Hoke A, Ignagni AR, Onders RP, Maragakis NJ
(2010) Peripheral hyperstimulation alters site of disease onset and course in SOD1 rats. Neurobiol Dis 39(3):252-64
Neuroscience
Zalfa C, et al.
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Zalfa C, Rota Nodari L, Vacchi E, Gelati M, Profico D, Boido M, Binda E, De Filippis L, Copetti M, Garlatti V, Daniele P, Rosati J, De Luca A, Pinos F, Cajola L, Visioli A, Mazzini L, Vercelli A, Svelto M, Vescovi AL, Ferrari D
(2019) Transplantation of clinical-grade human neural stem cells reduces neuroinflammation, prolongs survival and delays disease progression in the SOD1 rats. Cell Death Dis 10(5):345
Neuroscience, Stem Cells/Regenerative Medicine/Tissue Engineering
Popović-Bijelić A, et al.
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Popović-Bijelić A, Mojović M, Stamenković S, Jovanović M, Selaković V, Andjus P, Bačić G
(2016) Iron-sulfur cluster damage by the superoxide radical in neural tissues of the SOD1(G93A) ALS rat model. Free Radic. Biol. Med. 96:313-22
Neuroscience
Lepore AC, et al.
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Lepore AC, Rauck B, Dejea C, Pardo AC, Rao MS, Rothstein JD, Maragakis NJ
(2008) Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease. Nat. Neurosci. 11(11):1294-301
Neuroscience
Bataveljić D, et al.
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Bataveljić D, Nikolić L, Milosević M, Todorović N, Andjus PR
(2012) Changes in the astrocytic aquaporin-4 and inwardly rectifying potassium channel expression in the brain of the amyotrophic lateral sclerosis SOD1(G93A) rat model. Glia 60(12):1991-2003
Neuroscience
Thomsen GM, et al.
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Thomsen GM, Gowing G, Latter J, Chen M, Vit JP, Staggenborg K, Avalos P, Alkaslasi M, Ferraiuolo L, Likhite S, Kaspar BK, Svendsen CN
(2014) Delayed disease onset and extended survival in the SOD1G93A rat model of amyotrophic lateral sclerosis after suppression of mutant SOD1 in the motor cortex. J. Neurosci. 34(47):15587-600
Neuroscience
Howland DS, et al.
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Howland DS, Liu J, She Y, Goad B, Maragakis NJ, Kim B, Erickson J, Kulik J, DeVito L, Psaltis G, DeGennaro LJ, Cleveland DW, Rothstein JD
(2002) Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc. Natl. Acad. Sci. U.S.A. 99(3):1604-9
Neuroscience
Storkebaum E, et al.
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Storkebaum E, Lambrechts D, Dewerchin M, Moreno-Murciano MP, Appelmans S, Oh H, Van Damme P, Rutten B, Man WY, De Mol M, Wyns S, Manka D, Vermeulen K, Van Den Bosch L, Mertens N, Schmitz C, Robberecht W, Conway EM, Collen D, Moons L, Carmeliet P
(2005) Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS. Nat. Neurosci. 8(1):85-92
Neuroscience
Dunlop J, et al.
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Dunlop J, Beal McIlvain H, She Y, Howland DS
(2003) Impaired spinal cord glutamate transport capacity and reduced sensitivity to riluzole in a transgenic superoxide dismutase mutant rat model of amyotrophic lateral sclerosis. J. Neurosci. 23(5):1688-96
Neuroscience
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