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Search results 101 to 200 out of 487 for Smn1

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Category: Publication
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Type Details Score
Publication
29982416 | J:265815
First Author: Iyer CC
Year: 2018
Journal: Hum Mol Genet
Title: Mild SMN missense alleles are only functional in the presence of SMN2 in mammals.
Volume: 27
Issue: 19
Pages: 3404-3416
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Publication
26931466 | J:348120
First Author: Zhao X
Year: 2016
Journal: Hum Mol Genet
Title: Pharmacokinetics, pharmacodynamics, and efficacy of a small-molecule SMN2 splicing modifier in mouse models of spinal muscular atrophy.
Volume: 25
Issue: 10
Pages: 1885-1899
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Publication
32038174 | J:314870
 
First Author: Gatius A
Year: 2019
Journal: Front Cell Neurosci
Title: The Y172 Monoclonal Antibody Against p-c-Jun (Ser63) Is a Marker of the Postsynaptic Compartment of C-Type Cholinergic Afferent Synapses on Motoneurons.
Volume: 13
Pages: 582
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Publication
31828288 | J:293410
First Author: Kannan A
Year: 2020
Journal: Brain
Title: ZPR1 prevents R-loop accumulation, upregulates SMN2 expression and rescues spinal muscular atrophy.
Volume: 143
Issue: 1
Pages: 69-93
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Publication
26134627 | J:238311
First Author: McGovern VL
Year: 2015
Journal: PLoS One
Title: Plastin 3 Expression Does Not Modify Spinal Muscular Atrophy Severity in the ∆7 SMA Mouse.
Volume: 10
Issue: 7
Pages: e0132364
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Publication
23029491 | J:191961
First Author: Lee AJ
Year: 2012
Journal: PLoS One
Title: Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy.
Volume: 7
Issue: 9
Pages: e46353
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Publication
24781211 | J:213594
First Author: Osman EY
Year: 2014
Journal: Hum Mol Genet
Title: Morpholino antisense oligonucleotides targeting intronic repressor Element1 improve phenotype in SMA mouse models.
Volume: 23
Issue: 18
Pages: 4832-45
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Publication
24086650 | J:206015
First Author: Paez-Colasante X
Year: 2013
Journal: PLoS One
Title: Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons.
Volume: 8
Issue: 9
Pages: e75866
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Publication
23512182 | J:213313
First Author: Taylor AS
Year: 2013
Journal: Transgenic Res
Title: Restoration of SMN to Emx-1 expressing cortical neurons is not sufficient to provide benefit to a severe mouse model of Spinal Muscular Atrophy.
Volume: 22
Issue: 5
Pages: 1029-36
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Publication
20019802 | J:161744
First Author: Bäumer D
Year: 2009
Journal: PLoS Genet
Title: Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy.
Volume: 5
Issue: 12
Pages: e1000773
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Publication
31851921 | J:293107
First Author: Simon CM
Year: 2019
Journal: Cell Rep
Title: Stasimon Contributes to the Loss of Sensory Synapses and Motor Neuron Death in a Mouse Model of Spinal Muscular Atrophy.
Volume: 29
Issue: 12
Pages: 3885-3901.e5
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Publication
21785219 | J:176007
First Author: Lutz CM
Year: 2011
Journal: J Clin Invest
Title: Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.
Volume: 121
Issue: 8
Pages: 3029-41
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Publication
24760765 | J:213599
First Author: Bricceno KV
Year: 2014
Journal: Hum Mol Genet
Title: Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics.
Volume: 23
Issue: 18
Pages: 4745-57
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Publication
22723710 | J:185657
First Author: Martinez TL
Year: 2012
Journal: J Neurosci
Title: Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy.
Volume: 32
Issue: 25
Pages: 8703-15
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Publication
29281826 | J:277586
First Author: Simon CM
Year: 2017
Journal: Cell Rep
Title: Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy.
Volume: 21
Issue: 13
Pages: 3767-3780
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Publication
28504671 | J:244585
First Author: Fletcher EV
Year: 2017
Journal: Nat Neurosci
Title: Reduced sensory synaptic excitation impairs motor neuron function via Kv2.1 in spinal muscular atrophy.
Volume: 20
Issue: 7
Pages: 905-916
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Publication
37678880 | J:355156
First Author: Delestrée N
Year: 2023
Journal: Brain
Title: Serotonergic dysfunction impairs locomotor coordination in spinal muscular atrophy.
Volume: 146
Issue: 11
Pages: 4574-4593
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31801075 | J:299957
First Author: Vukojicic A
Year: 2019
Journal: Cell Rep
Title: The Classical Complement Pathway Mediates Microglia-Dependent Remodeling of Spinal Motor Circuits during Development and in SMA.
Volume: 29
Issue: 10
Pages: 3087-3100.e7
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Publication
33504650 | J:302153
 
First Author: Kong L
Year: 2021
Journal: Sci Transl Med
Title: Impaired prenatal motor axon development necessitates early therapeutic intervention in severe SMA.
Volume: 13
Issue: 578
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Publication
26206889 | J:226038
First Author: McGovern VL
Year: 2015
Journal: Hum Mol Genet
Title: SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA.
Volume: 24
Issue: 19
Pages: 5524-41
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Publication
28570645 | J:245188
First Author: Li W
Year: 2017
Journal: PLoS One
Title: How do SMA-linked mutations of SMN1 lead to structural/functional deficiency of the SMA protein?
Volume: 12
Issue: 6
Pages: e0178519
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Publication
21389246 | J:170079
First Author: Fallini C
Year: 2011
Journal: J Neurosci
Title: The survival of motor neuron (SMN) protein interacts with the mRNA-binding protein HuD and regulates localization of poly(A) mRNA in primary motor neuron axons.
Volume: 31
Issue: 10
Pages: 3914-25
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Publication
25911097 | J:238443
First Author: Workman E
Year: 2015
Journal: J Biol Chem
Title: Gemin5 Binds to the Survival Motor Neuron mRNA to Regulate SMN Expression.
Volume: 290
Issue: 25
Pages: 15662-9
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Publication
9070939 | J:38576
First Author: Viollet L
Year: 1997
Journal: Genomics
Title: cDNA isolation, expression, and chromosomal localization of the mouse survival motor neuron gene (Smn).
Volume: 40
Issue: 1
Pages: 185-8
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9275227 | J:42813
First Author: Schrank B
Year: 1997
Journal: Proc Natl Acad Sci U S A
Title: Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos.
Volume: 94
Issue: 18
Pages: 9920-5
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24067532 | J:206033
First Author: Sleigh JN
Year: 2014
Journal: Hum Mol Genet
Title: Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy.
Volume: 23
Issue: 4
Pages: 855-69
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Publication
18680723 | J:140130
First Author: Rose FF Jr
Year: 2008
Journal: Biochem Biophys Res Commun
Title: The Wallerian degeneration slow (Wld(s)) gene does not attenuate disease in a mouse model of spinal muscular atrophy.
Volume: 375
Issue: 1
Pages: 119-23
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Publication
10749994 | J:61396
First Author: Frugier T
Year: 2000
Journal: Hum Mol Genet
Title: Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy.
Volume: 9
Issue: 5
Pages: 849-58
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Publication
15076752 | J:89836
First Author: Ferri A
Year: 2004
Journal: Neuroreport
Title: Progressive and selective degeneration of motoneurons in a mouse model of SMA.
Volume: 15
Issue: 2
Pages: 275-80
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Publication
16118268 | J:356612
First Author: Olaso R
Year: 2006
Journal: Physiol Genomics
Title: Activation of RNA metabolism-related genes in mouse but not human tissues deficient in SMN.
Volume: 24
Issue: 2
Pages: 97-104
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22198571 | J:182347
First Author: Donnelly EM
Year: 2012
Journal: Neurobiol Dis
Title: Characterization of a murine model of SMA.
Volume: 45
Issue: 3
Pages: 992-8
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15509541 | J:93626
First Author: Vitte JM
Year: 2004
Journal: Am J Pathol
Title: Deletion of murine Smn exon 7 directed to liver leads to severe defect of liver development associated with iron overload.
Volume: 165
Issue: 5
Pages: 1731-41
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Publication
31402618 | J:355139
First Author: Deguise MO
Year: 2019
Journal: Ann Clin Transl Neurol
Title: Abnormal fatty acid metabolism is a core component of spinal muscular atrophy.
Volume: 6
Issue: 8
Pages: 1519-1532
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28108555 | J:241662
First Author: Deguise MO
Year: 2017
Journal: Hum Mol Genet
Title: Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice.
Volume: 26
Issue: 4
Pages: 801-819
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31365319 | J:296326
First Author: Quinlan KA
Year: 2019
Journal: J Neurophysiol
Title: Hyperexcitability precedes motoneuron loss in the Smn2B/- mouse model of spinal muscular atrophy.
Volume: 122
Issue: 4
Pages: 1297-1311
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20085811 | J:159930
First Author: Michaud M
Year: 2010
Journal: Neurobiol Dis
Title: Neuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy.
Volume: 38
Issue: 1
Pages: 125-35
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21693563 | J:174791
First Author: Kwon DY
Year: 2011
Journal: Hum Mol Genet
Title: Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice.
Volume: 20
Issue: 18
Pages: 3667-77
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Publication
31139093 | J:290196
 
First Author: Qian X
Year: 2019
Journal: Front Physiol
Title: Survival Motor Neuron (SMN) Protein Insufficiency Exacerbates Renal Ischemia/Reperfusion Injury.
Volume: 10
Pages: 559
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Publication
28515487 | J:287440
First Author: Mirra A
Year: 2017
Journal: Sci Rep
Title: Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice.
Volume: 7
Issue: 1
Pages: 2033
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Publication
- | J:144853
     
First Author: Bordet T
Year: 2009
Journal: MGI Direct Data Submission
Title: Generation of an SMN2 transgene (line 46)
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Publication
- | J:144852
     
First Author: Bordet T
Year: 2009
Journal: MGI Direct Data Submission
Title: Generation of an SMN2 transgene (line 11)
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Publication
27349908 | J:267811
 
First Author: Deguise MO
Year: 2016
Journal: Sci Rep
Title: Differential induction of muscle atrophy pathways in two mouse models of spinal muscular atrophy.
Volume: 6
Pages: 28846
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12091709 | J:81784
First Author: Jablonka S
Year: 2002
Journal: Proc Natl Acad Sci U S A
Title: Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death.
Volume: 99
Issue: 15
Pages: 10126-31
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Publication
11238465 | J:67884
First Author: Cifuentes-Diaz C
Year: 2001
Journal: J Cell Biol
Title: Deletion of murine SMN exon 7 directed to skeletal muscle leads to severe muscular dystrophy.
Volume: 152
Issue: 5
Pages: 1107-14
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Publication
12743106 | J:83343
First Author: Nicole S
Year: 2003
Journal: J Cell Biol
Title: Intact satellite cells lead to remarkable protection against Smn gene defect in differentiated skeletal muscle.
Volume: 161
Issue: 3
Pages: 571-82
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22644669 | J:194656
First Author: Piccoli M
Year: 2012
Journal: Stem Cells
Title: Amniotic fluid stem cells restore the muscle cell niche in a HSA-Cre, Smn(F7/F7) mouse model.
Volume: 30
Issue: 8
Pages: 1675-84
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Publication
37498175 | J:343670
First Author: Brown SJ
Year: 2023
Journal: Hum Mol Genet
Title: AAV9-mediated SMN gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in Smn2B/- spinal muscular atrophy mice.
Volume: 32
Issue: 20
Pages: 2950-2965
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Publication
35159369 | J:321586
 
First Author: Leo M
Year: 2022
Journal: Cells
Title: Induction of Survival of Motor Neuron (SMN) Protein Deficiency in Spinal Astrocytes by Small Interfering RNA as an In Vitro Model of Spinal Muscular Atrophy.
Volume: 11
Issue: 3
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Publication
27698380 | J:266714
 
First Author: Szunyogova E
Year: 2016
Journal: Sci Rep
Title: Survival Motor Neuron (SMN) protein is required for normal mouse liver development.
Volume: 6
Pages: 34635
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27557711 | J:246582
First Author: Doktor TK
Year: 2017
Journal: Nucleic Acids Res
Title: RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns.
Volume: 45
Issue: 1
Pages: 395-416
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27699224 | J:290245
First Author: Powis RA
Year: 2016
Journal: JCI Insight
Title: Systemic restoration of UBA1 ameliorates disease in spinal muscular atrophy.
Volume: 1
Issue: 11
Pages: e87908
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Publication
36099045 | J:331574
 
First Author: Zhou H
Year: 2022
Journal: J Clin Invest
Title: Microvasculopathy in spinal muscular atrophy is driven by a reversible autonomous endothelial cell defect.
Volume: 132
Issue: 21
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30239612 | J:355103
First Author: Shorrock HK
Year: 2018
Journal: Brain
Title: UBA1/GARS-dependent pathways drive sensory-motor connectivity defects in spinal muscular atrophy.
Volume: 141
Issue: 10
Pages: 2878-2894
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25645699 | J:355104
First Author: Staropoli JF
Year: 2015
Journal: Genomics
Title: Rescue of gene-expression changes in an induced mouse model of spinal muscular atrophy by an antisense oligonucleotide that promotes inclusion of SMN2 exon 7.
Volume: 105
Issue: 4
Pages: 220-8
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29961886 | J:355100
First Author: Janzen E
Year: 2018
Journal: Brain
Title: CHP1 reduction ameliorates spinal muscular atrophy pathology by restoring calcineurin activity and endocytosis.
Volume: 141
Issue: 8
Pages: 2343-2361
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28916199 | J:258965
 
First Author: Hensel N
Year: 2017
Journal: Neurobiol Dis
Title: ERK and ROCK functionally interact in a signaling network that is compensationally upregulated in Spinal Muscular Atrophy.
Volume: 108
Pages: 352-361
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20624852 | J:355105
First Author: Hua Y
Year: 2010
Journal: Genes Dev
Title: Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model.
Volume: 24
Issue: 15
Pages: 1634-44
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22504300 | J:355108
First Author: Rigo F
Year: 2012
Journal: Nat Chem Biol
Title: Synthetic oligonucleotides recruit ILF2/3 to RNA transcripts to modulate splicing.
Volume: 8
Issue: 6
Pages: 555-61
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36835624 | J:336315
 
First Author: Muiños-Bühl A
Year: 2023
Journal: Int J Mol Sci
Title: Long-Term SMN- and Ncald-ASO Combinatorial Therapy in SMA Mice and NCALD-ASO Treatment in hiPSC-Derived Motor Neurons Show Protective Effects.
Volume: 24
Issue: 4
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26647309 | J:229319
First Author: Tsai LK
Year: 2016
Journal: Hum Mol Genet
Title: Hypothermia improves disease manifestations in SMA mice via SMN augmentation.
Volume: 25
Issue: 4
Pages: 631-41
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33693569 | J:307088
First Author: Lopez-Manzaneda M
Year: 2021
Journal: Hum Mol Genet
Title: Calcium is reduced in presynaptic mitochondria of motor nerve terminals during neurotransmission in SMA mice.
Volume: 30
Issue: 8
Pages: 629-643
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33575118 | J:355117
 
First Author: Winkelsas AM
Year: 2021
Journal: Mol Ther Nucleic Acids
Title: Targeting the 5' untranslated region of SMN2 as a therapeutic strategy for spinal muscular atrophy.
Volume: 23
Pages: 731-742
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26576026 | J:257101
First Author: Powis RA
Year: 2016
Journal: J Anat
Title: Selective loss of alpha motor neurons with sparing of gamma motor neurons and spinal cord cholinergic neurons in a mouse model of spinal muscular atrophy.
Volume: 228
Issue: 3
Pages: 443-51
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27163330 | J:252462
First Author: Sintusek P
Year: 2016
Journal: PLoS One
Title: Histopathological Defects in Intestine in Severe Spinal Muscular Atrophy Mice Are Improved by Systemic Antisense Oligonucleotide Treatment.
Volume: 11
Issue: 5
Pages: e0155032
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27736905 | J:355037
First Author: Custer SK
Year: 2016
Journal: PLoS One
Title: Altered mRNA Splicing in SMN-Depleted Motor Neuron-Like Cells.
Volume: 11
Issue: 10
Pages: e0163954
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31019235 | J:281582
First Author: Qu R
Year: 2019
Journal: Sci Rep
Title: SMN deficiency causes pain hypersensitivity in a mild SMA mouse model through enhancing excitability of nociceptive dorsal root ganglion neurons.
Volume: 9
Issue: 1
Pages: 6493
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32103257 | J:286618
First Author: Sheng L
Year: 2020
Journal: Nucleic Acids Res
Title: Comparison of the efficacy of MOE and PMO modifications of systemic antisense oligonucleotides in a severe SMA mouse model.
Volume: 48
Issue: 6
Pages: 2853-2865
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19961830 | J:156775
First Author: Gogliotti RG
Year: 2010
Journal: Biochem Biophys Res Commun
Title: Molecular and phenotypic reassessment of an infrequently used mouse model for spinal muscular atrophy.
Volume: 391
Issue: 1
Pages: 517-22
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32644120 | J:296437
First Author: Motyl AAL
Year: 2020
Journal: Hum Mol Genet
Title: Pre-natal manifestation of systemic developmental abnormalities in spinal muscular atrophy.
Volume: 29
Issue: 16
Pages: 2674-2683
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36074806 | J:355040
First Author: Sun J
Year: 2022
Journal: PLoS Genet
Title: Single-cell RNA sequencing reveals dysregulation of spinal cord cell types in a severe spinal muscular atrophy mouse model.
Volume: 18
Issue: 9
Pages: e1010392
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38470509 | J:355191
First Author: Roos A
Year: 2024
Journal: Acta Neuropathol
Title: Alteration of LARGE1 abundance in patients and a mouse model of 5q-associated spinal muscular atrophy.
Volume: 147
Issue: 1
Pages: 53
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22348054 | J:185230
First Author: Hensel N
Year: 2012
Journal: PLoS One
Title: Analysis of the fibroblast growth factor system reveals alterations in a mouse model of spinal muscular atrophy.
Volume: 7
Issue: 2
Pages: e31202
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16107648 | J:101180
First Author: Grondard C
Year: 2005
Journal: J Neurosci
Title: Regular exercise prolongs survival in a type 2 spinal muscular atrophy model mouse.
Volume: 25
Issue: 33
Pages: 7615-22
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25583329 | J:355157
First Author: Hua Y
Year: 2015
Journal: Genes Dev
Title: Motor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse models.
Volume: 29
Issue: 3
Pages: 288-97
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28426667 | J:242984
First Author: Boyd PJ
Year: 2017
Journal: PLoS Genet
Title: Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy.
Volume: 13
Issue: 4
Pages: e1006744
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23073311 | J:355153
First Author: Schreml J
Year: 2013
Journal: Eur J Hum Genet
Title: Severe SMA mice show organ impairment that cannot be rescued by therapy with the HDACi JNJ-26481585.
Volume: 21
Issue: 6
Pages: 643-52
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24784568 | J:355158
First Author: Rigo F
Year: 2014
Journal: J Pharmacol Exp Ther
Title: Pharmacology of a central nervous system delivered 2'-O-methoxyethyl-modified survival of motor neuron splicing oligonucleotide in mice and nonhuman primates.
Volume: 350
Issue: 1
Pages: 46-55
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27124114 | J:253538
First Author: Lin TL
Year: 2016
Journal: PLoS One
Title: Selective Neuromuscular Denervation in Taiwanese Severe SMA Mouse Can Be Reversed by Morpholino Antisense Oligonucleotides.
Volume: 11
Issue: 4
Pages: e0154723
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31230718 | J:325210
First Author: Torres-Benito L
Year: 2019
Journal: Am J Hum Genet
Title: NCALD Antisense Oligonucleotide Therapy in Addition to Nusinersen further Ameliorates Spinal Muscular Atrophy in Mice.
Volume: 105
Issue: 1
Pages: 221-230
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27170316 | J:236986
First Author: Hunter G
Year: 2016
Journal: Hum Mol Genet
Title: Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy.
Volume: 25
Issue: 13
Pages: 2853-2861
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Publication
22930275 | J:203026
First Author: Biondi O
Year: 2012
Journal: J Physiol
Title: Physical exercise reduces cardiac defects in type 2 spinal muscular atrophy-like mice.
Volume: 590
Issue: 22
Pages: 5907-25
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Publication
23583590 | J:200043
 
First Author: Somers E
Year: 2013
Journal: Neurosci Lett
Title: Increasing SMN levels using the histone deacetylase inhibitor SAHA ameliorates defects in skeletal muscle microvasculature in a mouse model of severe spinal muscular atrophy.
Volume: 544
Pages: 100-4
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Publication
29069603 | J:254196
First Author: Bernabò P
Year: 2017
Journal: Cell Rep
Title: In Vivo Translatome Profiling in Spinal Muscular Atrophy Reveals a Role for SMN Protein in Ribosome Biology.
Volume: 21
Issue: 4
Pages: 953-965
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Publication
36719755 | J:334923
 
First Author: Aslesh T
Year: 2023
Journal: JCI Insight
Title: DG9-conjugated morpholino rescues phenotype in SMA mice by reaching the CNS via a subcutaneous administration.
Volume: 8
Issue: 5
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Publication
33931501 | J:309198
 
First Author: Hensel N
Year: 2021
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