| Year |
Citation |
Score |
| 2022 |
Comley LH, Kline RA, Thomson AK, Woschitz V, Landeros EV, Osman EY, Lorson CL, Murray LM. Motor Unit Recovery Following Smn Restoration in Mouse Models of Spinal Muscular Atrophy. Human Molecular Genetics. PMID 35551393 DOI: 10.1093/hmg/ddac097 |
0.406 |
|
| 2022 |
Dumas SA, Villalón E, Bergman EM, Wilson KJ, Marugan JJ, Lorson CL, Burnett BG. A combinatorial approach increases SMN level in SMA model mice. Human Molecular Genetics. PMID 35419606 DOI: 10.1093/hmg/ddac068 |
0.452 |
|
| 2022 |
Tang Z, Hegde S, Zhao J, Zhu S, Johnson KA, Lorson CL, Wang J. CRISPR-mediated Enzyme Fragment Complementation Assay for Quantification of the Stability of Splice Isoforms. Chembiochem : a European Journal of Chemical Biology. PMID 35235240 DOI: 10.1002/cbic.202200012 |
0.338 |
|
| 2021 |
Smith CE, Lorson MA, Ricardez Hernandez SM, Al Rawi Z, Mao J, Marquez J, Villalón E, Keilholz AN, Smith CL, Garro-Kacher MO, Morcos T, Davis DJ, Bryda EC, Nichols NL, Lorson CL. The Ighmbp2D564N mouse model is the first SMARD1 model to demonstrate respiratory defects. Human Molecular Genetics. PMID 34726235 DOI: 10.1093/hmg/ddab317 |
0.349 |
|
| 2021 |
Shababi M, Smith CE, Ricardez Hernandez SM, Marquez J, Al Rawi Z, Villalón E, Farris KD, Garro-Kacher MO, Lorson CL. Defining the optimal dose and therapeutic window in SMA with respiratory distress type I model mice, FVB/NJ- . Molecular Therapy. Methods & Clinical Development. 23: 23-32. PMID 34553000 DOI: 10.1016/j.omtm.2021.07.008 |
0.417 |
|
| 2021 |
McCormack NM, Villalón E, Viollet C, Soltis AR, Dalgard CL, Lorson CL, Burnett BG. Survival motor neuron deficiency slows myoblast fusion through reduced myomaker and myomixer expression. Journal of Cachexia, Sarcopenia and Muscle. PMID 34115448 DOI: 10.1002/jcsm.12740 |
0.436 |
|
| 2021 |
Rietz A, Hodgetts KJ, Lusic H, Quist KM, Osman EY, Lorson CL, Androphy EJ. Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophy. Life Science Alliance. 4. PMID 33234679 DOI: 10.26508/lsa.202000889 |
0.45 |
|
| 2020 |
Kaifer KA, Villalón E, Smith CE, Simon ME, Marquez J, Hopkins AE, Morcos TI, Lorson CL. AAV9-DOK7 gene therapy reduces disease severity in Smn SMA model mice. Biochemical and Biophysical Research Communications. 530: 107-114. PMID 32828271 DOI: 10.1016/J.Bbrc.2020.07.031 |
0.527 |
|
| 2020 |
Osman EY, Van Alstyne M, Yen PF, Lotti F, Feng Z, Ling KK, Ko CP, Pellizzoni L, Lorson CL. Minor snRNA gene delivery improves the loss of proprioceptive synapses on SMA motor neurons. Jci Insight. PMID 32516136 DOI: 10.1172/Jci.Insight.130574 |
0.595 |
|
| 2019 |
Shababi M, Smith CE, Kacher M, Alrawi Z, Villalón E, Davis D, Bryda EC, Lorson CL. Development of a novel severe mouse model of spinal muscular atrophy with respiratory distress type 1: FVB-nmd. Biochemical and Biophysical Research Communications. PMID 31604525 DOI: 10.1016/J.Bbrc.2019.10.032 |
0.516 |
|
| 2019 |
Villalón E, Kline RA, Smith CE, Lorson ZC, Osman EY, O'Day S, Murray LM, Lorson CL. AAV9-Stathmin1 gene delivery improves disease phenotype in an intermediate mouse model of Spinal Muscular Atrophy. Human Molecular Genetics. PMID 31363739 DOI: 10.1093/Hmg/Ddz188 |
0.577 |
|
| 2019 |
Osman EY, Bolding MR, Villalón E, Kaifer KA, Lorson ZC, Tisdale S, Hao Y, Conant GC, Pires JC, Pellizzoni L, Lorson CL. Functional characterization of SMN evolution in mouse models of SMA. Scientific Reports. 9: 9472. PMID 31263170 DOI: 10.1038/S41598-019-45822-8 |
0.588 |
|
| 2019 |
Villalón E, Lee NN, Marquez J, Lorson CL. Muscle fiber-type selective propensity to pathology in the nmd mouse model of SMARD1. Biochemical and Biophysical Research Communications. 516: 313-319. PMID 31256932 DOI: 10.1016/J.Bbrc.2019.06.117 |
0.362 |
|
| 2019 |
Kaifer KA, Villalón E, O'Brien BS, Sison SL, Smith CE, Simon ME, Marquez J, O'Day S, Hopkins AE, Neff R, Rindt H, Ebert AD, Lorson CL. AAV9-Mediated Delivery of miR-23a Reduces Disease Severity in Smn2B-/SMA Model Mice. Human Molecular Genetics. PMID 31211843 DOI: 10.1093/Hmg/Ddz142 |
0.372 |
|
| 2019 |
Osman EY, Rietz A, Kline RA, Cherry JJ, Hodgetts KJ, Lorson CL, Androphy EJ. Intraperitoneal delivery of a novel drug-like compound improves disease severity in severe and intermediate mouse models of Spinal Muscular Atrophy. Scientific Reports. 9: 1633. PMID 30733501 DOI: 10.1038/s41598-018-38208-9 |
0.543 |
|
| 2018 |
Shababi M, Villalón E, Kaifer KA, DeMarco V, Lorson CL. A Direct Comparison of IV and ICV Delivery Methods for Gene Replacement Therapy in a Mouse Model of SMARD1. Molecular Therapy. Methods & Clinical Development. 10: 348-360. PMID 30202772 DOI: 10.1016/J.Omtm.2018.08.005 |
0.469 |
|
| 2017 |
Gray KM, Kaifer KA, Baillat D, Wen Y, Bonacci TR, Ebert AD, Raimer AC, Spring AM, Have ST, Glascock JJ, Gupta K, Van Duyne GD, Emanuele MJ, Lamond AI, Wagner EJ, ... Lorson CL, et al. Self-oligomerization regulates stability of Survival Motor Neuron (SMN) protein isoforms by sequestering an SCF(Slmb) degron. Molecular Biology of the Cell. PMID 29167380 DOI: 10.1091/Mbc.E17-11-0627 |
0.546 |
|
| 2017 |
Choi S, Calder AN, Miller EH, Anderson KP, Fiejtek DK, Rietz A, Li H, Cherry JJ, Quist KM, Xing X, Glicksman MA, Cuny GD, Lorson CL, Androphy EA, Hodgetts KJ. Optimization of a series of heterocycles as survival motor neuron gene transcription enhancers. Bioorganic & Medicinal Chemistry Letters. PMID 29103974 DOI: 10.1016/J.Bmcl.2017.10.066 |
0.448 |
|
| 2017 |
Rindt H, Tom CM, Lorson CL, Mattis VB. Optimization of trans-Splicing for Huntington's Disease RNA Therapy. Frontiers in Neuroscience. 11: 544. PMID 29066943 DOI: 10.3389/Fnins.2017.00544 |
0.758 |
|
| 2017 |
Sison SL, Patitucci TN, Seminary ER, Villalon E, Lorson CL, Ebert AD. Astrocyte-produced miR-146a as a mediator of motor neuron loss in spinal muscular atrophy. Human Molecular Genetics. PMID 28637335 DOI: 10.1093/Hmg/Ddx230 |
0.347 |
|
| 2017 |
Osman EY, Washington CW, Simon ME, Megiddo D, Greif DH, Lorson CL. Analysis of Azithromycin Monohydrate as a Single or a Combinatorial Therapy in a Mouse Model of Severe Spinal Muscular Atrophy. Journal of Neuromuscular Diseases. PMID 28598854 DOI: 10.3233/JND-170230 |
0.465 |
|
| 2017 |
Rietz A, Li H, Quist KM, Cherry JJ, Lorson CL, Burnett B, Kern NL, Calder AN, Fritsche M, Lusic H, Boaler PJ, Choi S, Xing X, Glicksman MA, Cuny GD, et al. Discovery of a Small Molecule Probe that Post-translationally Stabilizes the Survival Motor Neuron Protein for the Treatment of Spinal Muscular Atrophy. Journal of Medicinal Chemistry. PMID 28481536 DOI: 10.1021/Acs.Jmedchem.6B01885 |
0.394 |
|
| 2017 |
Kline RA, Kaifer KA, Osman EY, Carella F, Tiberi A, Ross J, Pennetta G, Lorson CL, Murray LM. Comparison of independent screens on differentially vulnerable motor neurons reveals alpha-synuclein as a common modifier in motor neuron diseases. Plos Genetics. 13: e1006680. PMID 28362802 DOI: 10.1371/journal.pgen.1006680 |
0.381 |
|
| 2017 |
Kaifer KA, Villalón E, Osman EY, Glascock JJ, Arnold LL, Cornelison DD, Lorson CL. Plastin-3 extends survival and reduces severity in mouse models of spinal muscular atrophy. Jci Insight. 2: e89970. PMID 28289706 DOI: 10.1172/jci.insight.89970 |
0.519 |
|
| 2017 |
Khairallah MT, Astroski J, Custer SK, Androphy EJ, Franklin CL, Lorson CL. SMN deficiency negatively impacts red pulp macrophages and spleen development in mouse models of Spinal Muscular Atrophy. Human Molecular Genetics. PMID 28062667 DOI: 10.1093/Hmg/Ddx008 |
0.429 |
|
| 2016 |
Osman EY, Washington CW, Kaifer KA, Mazzasette C, Patitucci TN, Florea KM, Simon ME, Ko CP, Ebert AD, Lorson CL. Optimization of Morpholino Antisense Oligonucleotides Targeting the Intronic Repressor Element1 in Spinal Muscular Atrophy. Molecular Therapy : the Journal of the American Society of Gene Therapy. PMID 27401142 DOI: 10.1038/Mt.2016.145 |
0.509 |
|
| 2016 |
Shababi M, Feng Z, Villalon E, Sibigtroth CM, Osman EY, Miller MR, Williams-Simon PA, Lombardi A, Sass TH, Atkinson AK, Garcia ML, Ko CP, Lorson CL. Rescue of a Mouse Model of Spinal Muscular Atrophy with Respiratory Distress type 1 (SMARD1) by AAV9-IGHMBP2 is Dose Dependent. Molecular Therapy : the Journal of the American Society of Gene Therapy. PMID 26860981 DOI: 10.1038/Mt.2016.33 |
0.457 |
|
| 2015 |
Van Granigen Caesar G, Dale JM, Osman EY, Garcia ML, Lorson CL, Schulz LC. Placental Development in a Mouse Model of Spinal Muscular Atrophy. Biochemical and Biophysical Research Communications. PMID 26748185 DOI: 10.1016/j.bbrc.2015.12.120 |
0.349 |
|
| 2015 |
Rindt H, Feng Z, Mazzasette C, Glascock JJ, Valdivia D, Pyles N, Crawford TO, Swoboda KJ, Patitucci TN, Ebert AD, Sumner CJ, Ko CP, Lorson CL. Astrocytes influence the severity of spinal muscular atrophy. Human Molecular Genetics. 24: 4094-102. PMID 25911676 DOI: 10.1093/Hmg/Ddv148 |
0.555 |
|
| 2015 |
Seng CO, Magee C, Young PJ, Lorson CL, Allen JP. The SMN structure reveals its crucial role in snRNP assembly. Human Molecular Genetics. 24: 2138-46. PMID 25561692 DOI: 10.1093/Hmg/Ddu734 |
0.439 |
|
| 2014 |
Osman EY, Miller MR, Robbins KL, Lombardi AM, Atkinson AK, Brehm AJ, Lorson CL. Morpholino antisense oligonucleotides targeting intronic repressor Element1 improve phenotype in SMA mouse models. Human Molecular Genetics. 23: 4832-45. PMID 24781211 DOI: 10.1093/Hmg/Ddu198 |
0.539 |
|
| 2014 |
Robbins KL, Glascock JJ, Osman EY, Miller MR, Lorson CL. Defining the therapeutic window in a severe animal model of spinal muscular atrophy. Human Molecular Genetics. 23: 4559-68. PMID 24722206 DOI: 10.1093/Hmg/Ddu169 |
0.472 |
|
| 2014 |
Shababi M, Lorson CL, Rudnik-Schöneborn SS. Spinal muscular atrophy: a motor neuron disorder or a multi-organ disease? Journal of Anatomy. 224: 15-28. PMID 23876144 DOI: 10.1111/Joa.12083 |
0.534 |
|
| 2013 |
Cherry JJ, Osman EY, Evans MC, Choi S, Xing X, Cuny GD, Glicksman MA, Lorson CL, Androphy EJ. Enhancement of SMN protein levels in a mouse model of spinal muscular atrophy using novel drug-like compounds. Embo Molecular Medicine. 5: 1035-50. PMID 23740718 DOI: 10.1002/Emmm.201202305 |
0.593 |
|
| 2013 |
Taylor AS, Glascock JJ, Rose FF, Lutz C, Lorson CL. Restoration of SMN to Emx-1 expressing cortical neurons is not sufficient to provide benefit to a severe mouse model of Spinal Muscular Atrophy. Transgenic Research. 22: 1029-36. PMID 23512182 DOI: 10.1007/S11248-013-9702-Y |
0.552 |
|
| 2013 |
Cobb MS, Rose FF, Rindt H, Glascock JJ, Shababi M, Miller MR, Osman EY, Yen PF, Garcia ML, Martin BR, Wetz MJ, Mazzasette C, Feng Z, Ko CP, Lorson CL. Development and characterization of an SMN2-based intermediate mouse model of Spinal Muscular Atrophy. Human Molecular Genetics. 22: 1843-55. PMID 23390132 DOI: 10.1093/Hmg/Ddt037 |
0.613 |
|
| 2012 |
Lorson MA, Lorson CL. SMN-inducing compounds for the treatment of spinal muscular atrophy. Future Medicinal Chemistry. 4: 2067-84. PMID 23157239 DOI: 10.4155/Fmc.12.131 |
0.534 |
|
| 2012 |
Mattis VB, Tom Chang CW, Lorson CL. Analysis of a read-through promoting compound in a severe mouse model of spinal muscular atrophy. Neuroscience Letters. 525: 72-5. PMID 22819971 DOI: 10.1016/J.Neulet.2012.07.024 |
0.802 |
|
| 2012 |
Rindt H, Yen PF, Thebeau CN, Peterson TS, Weisman GA, Lorson CL. Replacement of huntingtin exon 1 by trans-splicing. Cellular and Molecular Life Sciences : Cmls. 69: 4191-204. PMID 22814437 DOI: 10.1007/S00018-012-1083-5 |
0.535 |
|
| 2012 |
Shababi M, Habibi J, Ma L, Glascock JJ, Sowers JR, Lorson CL. Partial restoration of cardio-vascular defects in a rescued severe model of spinal muscular atrophy. Journal of Molecular and Cellular Cardiology. 52: 1074-82. PMID 22285962 DOI: 10.1016/J.Yjmcc.2012.01.005 |
0.478 |
|
| 2012 |
Glascock JJ, Shababi M, Wetz MJ, Krogman MM, Lorson CL. Direct central nervous system delivery provides enhanced protection following vector mediated gene replacement in a severe model of spinal muscular atrophy. Biochemical and Biophysical Research Communications. 417: 376-81. PMID 22172949 DOI: 10.1016/J.Bbrc.2011.11.121 |
0.538 |
|
| 2012 |
Rindt H, Buckley DM, Vale SM, Krogman M, Rose FF, Garcia ML, Lorson CL. Transgenic inactivation of murine myostatin does not decrease the severity of disease in a model of Spinal Muscular Atrophy. Neuromuscular Disorders : Nmd. 22: 277-85. PMID 22079083 DOI: 10.1016/J.Nmd.2011.10.012 |
0.549 |
|
| 2012 |
Osman EY, Yen PF, Lorson CL. Bifunctional RNAs targeting the intronic splicing silencer N1 increase SMN levels and reduce disease severity in an animal model of spinal muscular atrophy. Molecular Therapy : the Journal of the American Society of Gene Therapy. 20: 119-26. PMID 22031236 DOI: 10.1038/Mt.2011.232 |
0.588 |
|
| 2012 |
Glascock JJ, Osman EY, Wetz MJ, Krogman MM, Shababi M, Lorson CL. Decreasing disease severity in symptomatic, Smn(-/-);SMN2(+/+), spinal muscular atrophy mice following scAAV9-SMN delivery. Human Gene Therapy. 23: 330-5. PMID 22029744 DOI: 10.1089/Hum.2011.166 |
0.592 |
|
| 2012 |
Shababi M, Lorson CL. Optimization of SMN trans-splicing through the analysis of SMN introns. Journal of Molecular Neuroscience : Mn. 46: 459-69. PMID 21826391 DOI: 10.1007/S12031-011-9614-3 |
0.605 |
|
| 2011 |
Glascock JJ, Osman EY, Coady TH, Rose FF, Shababi M, Lorson CL. Delivery of therapeutic agents through intracerebroventricular (ICV) and intravenous (IV) injection in mice. Journal of Visualized Experiments : Jove. PMID 21988897 DOI: 10.3791/2968 |
0.357 |
|
| 2011 |
Coady TH, Lorson CL. SMN in spinal muscular atrophy and snRNP biogenesis. Wiley Interdisciplinary Reviews. Rna. 2: 546-64. PMID 21957043 DOI: 10.1002/Wrna.76 |
0.541 |
|
| 2011 |
Dale JM, Shen H, Barry DM, Garcia VB, Rose FF, Lorson CL, Garcia ML. The spinal muscular atrophy mouse model, SMAΔ7, displays altered axonal transport without global neurofilament alterations. Acta Neuropathologica. 122: 331-41. PMID 21681521 DOI: 10.1007/S00401-011-0848-5 |
0.391 |
|
| 2011 |
Lorson MA, Spate LD, Samuel MS, Murphy CN, Lorson CL, Prather RS, Wells KD. Disruption of the Survival Motor Neuron (SMN) gene in pigs using ssDNA. Transgenic Research. 20: 1293-304. PMID 21350916 DOI: 10.1007/S11248-011-9496-8 |
0.56 |
|
| 2011 |
Peter CJ, Evans M, Thayanithy V, Taniguchi-Ishigaki N, Bach I, Kolpak A, Bassell GJ, Rossoll W, Lorson CL, Bao ZZ, Androphy EJ. The COPI vesicle complex binds and moves with survival motor neuron within axons. Human Molecular Genetics. 20: 1701-11. PMID 21300694 DOI: 10.1093/Hmg/Ddr046 |
0.465 |
|
| 2011 |
Shababi M, Glascock J, Lorson CL. Combination of SMN trans-splicing and a neurotrophic factor increases the life span and body mass in a severe model of spinal muscular atrophy. Human Gene Therapy. 22: 135-44. PMID 20804424 DOI: 10.1089/Hum.2010.114 |
0.651 |
|
| 2010 |
Shababi M, Mattis VB, Lorson CL. Therapeutics that directly increase SMN expression to treat spinal muscular atrophy. Drug News & Perspectives. 23: 475-82. PMID 21031163 DOI: 10.1358/Dnp.2010.23.8.1507295 |
0.817 |
|
| 2010 |
Shababi M, Habibi J, Yang HT, Vale SM, Sewell WA, Lorson CL. Cardiac defects contribute to the pathology of spinal muscular atrophy models. Human Molecular Genetics. 19: 4059-71. PMID 20696672 DOI: 10.1093/Hmg/Ddq329 |
0.483 |
|
| 2010 |
Lorson CL, Rindt H, Shababi M. Spinal muscular atrophy: mechanisms and therapeutic strategies. Human Molecular Genetics. 19: R111-8. PMID 20392710 DOI: 10.1093/Hmg/Ddq147 |
0.579 |
|
| 2010 |
Shaw DJ, Morse R, Todd AG, Eggleton P, Lorson CL, Young PJ. Identification of a self-association domain in the Ewing's sarcoma protein: a novel function for arginine-glycine-glycine rich motifs? Journal of Biochemistry. 147: 885-93. PMID 20211855 DOI: 10.1093/Jb/Mvq025 |
0.407 |
|
| 2010 |
Coady TH, Lorson CL. Trans-splicing-mediated improvement in a severe mouse model of spinal muscular atrophy. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 30: 126-30. PMID 20053895 DOI: 10.1523/Jneurosci.4489-09.2010 |
0.659 |
|
| 2010 |
Butchbach ME, Rose FF, Rhoades S, Marston J, McCrone JT, Sinnott R, Lorson CL. Effect of diet on the survival and phenotype of a mouse model for spinal muscular atrophy. Biochemical and Biophysical Research Communications. 391: 835-40. PMID 19945425 DOI: 10.1016/J.Bbrc.2009.11.148 |
0.503 |
|
| 2009 |
Mattis VB, Fosso MY, Chang CW, Lorson CL. Subcutaneous administration of TC007 reduces disease severity in an animal model of SMA. Bmc Neuroscience. 10: 142. PMID 19948047 DOI: 10.1186/1471-2202-10-142 |
0.81 |
|
| 2009 |
Le Roy F, Charton K, Lorson CL, Richard I. RNA-targeting approaches for neuromuscular diseases. Trends in Molecular Medicine. 15: 580-91. PMID 19906562 DOI: 10.1016/J.Molmed.2009.10.005 |
0.484 |
|
| 2009 |
Shaw DJ, Morse R, Todd AG, Eggleton P, Lorson CL, Young PJ. Identification of a tripartite import signal in the Ewing Sarcoma protein (EWS). Biochemical and Biophysical Research Communications. 390: 1197-201. PMID 19879242 DOI: 10.1016/J.Bbrc.2009.10.120 |
0.351 |
|
| 2009 |
Mattis VB, Ebert AD, Fosso MY, Chang CW, Lorson CL. Delivery of a read-through inducing compound, TC007, lessens the severity of a spinal muscular atrophy animal model. Human Molecular Genetics. 18: 3906-13. PMID 19625298 DOI: 10.1093/Hmg/Ddp333 |
0.806 |
|
| 2009 |
Baughan TD, Dickson A, Osman EY, Lorson CL. Delivery of bifunctional RNAs that target an intronic repressor and increase SMN levels in an animal model of spinal muscular atrophy. Human Molecular Genetics. 18: 1600-11. PMID 19228773 DOI: 10.1093/Hmg/Ddp076 |
0.8 |
|
| 2009 |
Ebert AD, Yu J, Rose FF, Mattis VB, Lorson CL, Thomson JA, Svendsen CN. Induced pluripotent stem cells from a spinal muscular atrophy patient. Nature. 457: 277-80. PMID 19098894 DOI: 10.1038/Nature07677 |
0.783 |
|
| 2009 |
Rose FF, Mattis VB, Rindt H, Lorson CL. Delivery of recombinant follistatin lessens disease severity in a mouse model of spinal muscular atrophy. Human Molecular Genetics. 18: 997-1005. PMID 19074460 DOI: 10.1093/Hmg/Ddn426 |
0.757 |
|
| 2008 |
Dickson A, Osman E, Lorson CL. A negatively acting bifunctional RNA increases survival motor neuron both in vitro and in vivo. Human Gene Therapy. 19: 1307-15. PMID 19848583 DOI: 10.1089/Hum.2008.067 |
0.646 |
|
| 2008 |
Coady TH, Baughan TD, Shababi M, Passini MA, Lorson CL. Development of a single vector system that enhances trans-splicing of SMN2 transcripts. Plos One. 3: e3468. PMID 18941511 DOI: 10.1371/Journal.Pone.0003468 |
0.787 |
|
| 2008 |
Mattis VB, Butchbach ME, Lorson CL. Detection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy. Journal of Neuroscience Methods. 175: 36-43. PMID 18771690 DOI: 10.1016/J.Jneumeth.2008.07.024 |
0.788 |
|
| 2008 |
Rose FF, Meehan PW, Coady TH, Garcia VB, Garcia ML, Lorson CL. The Wallerian degeneration slow (Wld(s)) gene does not attenuate disease in a mouse model of spinal muscular atrophy. Biochemical and Biophysical Research Communications. 375: 119-23. PMID 18680723 DOI: 10.1016/J.Bbrc.2008.07.130 |
0.527 |
|
| 2008 |
Lorson MA, Dickson AM, Shaw DJ, Todd AG, Young EC, Morse R, Wolstencroft C, Lorson CL, Young PJ. Identification and characterisation of a nuclear localisation signal in the SMN associated protein, Gemin4. Biochemical and Biophysical Research Communications. 375: 33-7. PMID 18675250 DOI: 10.1016/J.Bbrc.2008.07.113 |
0.503 |
|
| 2008 |
Lorson MA, Spate LD, Prather RS, Lorson CL. Identification and characterization of the porcine (Sus scrofa) survival motor neuron (SMN1) gene: an animal model for therapeutic studies. Developmental Dynamics : An Official Publication of the American Association of Anatomists. 237: 2268-78. PMID 18651653 DOI: 10.1002/Dvdy.21642 |
0.61 |
|
| 2008 |
Mattis VB, Bowerman M, Kothary R, Lorson CL. A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neuroscience Letters. 442: 54-8. PMID 18601974 DOI: 10.1016/J.Neulet.2008.06.059 |
0.788 |
|
| 2008 |
Sakla MS, Lorson CL. Induction of full-length survival motor neuron by polyphenol botanical compounds. Human Genetics. 122: 635-43. PMID 17962980 DOI: 10.1007/S00439-007-0441-0 |
0.608 |
|
| 2008 |
Novoyatleva T, Heinrich B, Tang Y, Benderska N, Butchbach ME, Lorson CL, Lorson MA, Ben-Dov C, Fehlbaum P, Bracco L, Burghes AH, Bollen M, Stamm S. Protein phosphatase 1 binds to the RNA recognition motif of several splicing factors and regulates alternative pre-mRNA processing. Human Molecular Genetics. 17: 52-70. PMID 17913700 DOI: 10.1093/Hmg/Ddm284 |
0.432 |
|
| 2007 |
Coady TH, Shababi M, Tullis GE, Lorson CL. Restoration of SMN function: delivery of a trans-splicing RNA re-directs SMN2 pre-mRNA splicing. Molecular Therapy : the Journal of the American Society of Gene Therapy. 15: 1471-8. PMID 17551501 DOI: 10.1038/Sj.Mt.6300222 |
0.588 |
|
| 2006 |
Carrel TL, McWhorter ML, Workman E, Zhang H, Wolstencroft EC, Lorson C, Bassell GJ, Burghes AH, Beattie CE. Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 26: 11014-22. PMID 17065443 DOI: 10.1523/Jneurosci.1637-06.2006 |
0.419 |
|
| 2006 |
Mattis VB, Rai R, Wang J, Chang CW, Coady T, Lorson CL. Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts. Human Genetics. 120: 589-601. PMID 16951947 DOI: 10.1007/S00439-006-0245-7 |
0.801 |
|
| 2006 |
Baughan T, Shababi M, Coady TH, Dickson AM, Tullis GE, Lorson CL. Stimulating full-length SMN2 expression by delivering bifunctional RNAs via a viral vector. Molecular Therapy : the Journal of the American Society of Gene Therapy. 14: 54-62. PMID 16580882 DOI: 10.1016/J.Ymthe.2006.01.012 |
0.794 |
|
| 2006 |
Coady TH, Shababi M, Lorson CL. 253. AAV Delivery of a Trans-Splicing RNA Re-Directs SMN2 Splicing and Results in Increased Full-Length SMN Molecular Therapy. 13: S97. DOI: 10.1016/J.Ymthe.2006.08.280 |
0.646 |
|
| 2005 |
Wolstencroft EC, Mattis V, Bajer AA, Young PJ, Lorson CL. A non-sequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein levels. Human Molecular Genetics. 14: 1199-210. PMID 15790598 DOI: 10.1093/Hmg/Ddi131 |
0.792 |
|
| 2005 |
Young PJ, Newman A, Jensen KT, Burger LR, Pintel DJ, Lorson CL. Minute virus of mice small non-structural protein NS2 localizes within, but is not required for the formation of, Smn-associated autonomous parvovirus-associated replication bodies. The Journal of General Virology. 86: 1009-14. PMID 15784894 DOI: 10.1099/Vir.0.80564-0 |
0.359 |
|
| 2004 |
Masson JF, Barnhart M, Battaglia TM, Morris GE, Nieman RA, Young PJ, Lorson CL, Booksh KS. Monitoring of recombinant survival motor neuron protein using fiber-optic surface plasmon resonance. The Analyst. 129: 855-9. PMID 15343403 DOI: 10.1039/B403885A |
0.447 |
|
| 2004 |
Francis JW, Figueiredo D, vanderSpek JC, Ayala LM, Kim YS, Remington MP, Young PJ, Lorson CL, Ikebe S, Fishman PS, Brown RH. A survival motor neuron:tetanus toxin fragment C fusion protein for the targeted delivery of SMN protein to neurons. Brain Research. 995: 84-96. PMID 14644474 DOI: 10.1016/J.Brainres.2003.09.063 |
0.529 |
|
| 2003 |
Young PJ, Francis JW, Lince D, Coon K, Androphy EJ, Lorson CL. The Ewing's sarcoma protein interacts with the Tudor domain of the survival motor neuron protein. Brain Research. Molecular Brain Research. 119: 37-49. PMID 14597228 DOI: 10.1016/J.Molbrainres.2003.08.011 |
0.526 |
|
| 2003 |
Monani UR, Pastore MT, Gavrilina TO, Jablonka S, Le TT, Andreassi C, DiCocco JM, Lorson C, Androphy EJ, Sendtner M, Podell M, Burghes AH. A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy. The Journal of Cell Biology. 160: 41-52. PMID 12515823 DOI: 10.1083/Jcb.200208079 |
0.552 |
|
| 2002 |
Wehner KA, Ayala L, Kim Y, Young PJ, Hosler BA, Lorson CL, Baserga SJ, Francis JW. Survival motor neuron protein in the nucleolus of mammalian neurons. Brain Research. 945: 160-73. PMID 12126878 DOI: 10.1016/S0006-8993(02)02750-6 |
0.554 |
|
| 2002 |
Young PJ, Jensen KT, Burger LR, Pintel DJ, Lorson CL. Minute virus of mice small nonstructural protein NS2 interacts and colocalizes with the Smn protein. Journal of Virology. 76: 6364-9. PMID 12021369 DOI: 10.1128/Jvi.76.12.6364-6369.2002 |
0.406 |
|
| 2002 |
Young PJ, Jensen KT, Burger LR, Pintel DJ, Lorson CL. Minute virus of mice NS1 interacts with the SMN protein, and they colocalize in novel nuclear bodies induced by parvovirus infection. Journal of Virology. 76: 3892-904. PMID 11907229 DOI: 10.1128/Jvi.76.8.3892-3904.2002 |
0.389 |
|
| 2002 |
Young PJ, DiDonato CJ, Hu D, Kothary R, Androphy EJ, Lorson CL. SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2 beta 1. Human Molecular Genetics. 11: 577-87. PMID 11875052 DOI: 10.1093/Hmg/11.5.577 |
0.497 |
|
| 2002 |
Young PJ, Day PM, Zhou J, Androphy EJ, Morris GE, Lorson CL. A direct interaction between the survival motor neuron protein and p53 and its relationship to spinal muscular atrophy. The Journal of Biological Chemistry. 277: 2852-9. PMID 11704667 DOI: 10.1074/Jbc.M108769200 |
0.507 |
|
| 2001 |
DiDonato CJ, Lorson CL, De Repentigny Y, Simard L, Chartrand C, Androphy EJ, Kothary R. Regulation of murine survival motor neuron (Smn) protein levels by modifying Smn exon 7 splicing. Human Molecular Genetics. 10: 2727-36. PMID 11726560 DOI: 10.1093/Hmg/10.23.2727 |
0.585 |
|
| 2001 |
Zhang M, Lorson C, Androphy E, Zhou J. An in vivo reporter system for measuring increased inclusion of exon 7 in SMN2 mRNA: potential therapy of SMA Gene Therapy. 8: 1532-1538. PMID 11704813 DOI: 10.1038/Sj.Gt.3301550 |
0.546 |
|
| 2000 |
Young PJ, Man NT, Lorson CL, Le TT, Androphy EJ, Burghes AH, Morris GE. The exon 2b region of the spinal muscular atrophy protein, SMN, is involved in self-association and SIP1 binding. Human Molecular Genetics. 9: 2869-77. PMID 11092763 DOI: 10.1093/Hmg/9.19.2869 |
0.537 |
|
| 2000 |
Hofmann Y, Lorson CL, Stamm S, Androphy EJ, Wirth B. Htra2-beta 1 stimulates an exonic splicing enhancer and can restore full-length SMN expression to survival motor neuron 2 (SMN2). Proceedings of the National Academy of Sciences of the United States of America. 97: 9618-23. PMID 10931943 DOI: 10.1073/Pnas.160181697 |
0.579 |
|
| 2000 |
Lorson CL, Androphy EJ. An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN. Human Molecular Genetics. 9: 259-65. PMID 10607836 DOI: 10.1093/Hmg/9.2.259 |
0.621 |
|
| 1999 |
Strasswimmer J, Lorson CL, Breiding DE, Chen JJ, Le T, Burghes AH, Androphy EJ. Identification of survival motor neuron as a transcriptional activator-binding protein. Human Molecular Genetics. 8: 1219-26. PMID 10369867 DOI: 10.1093/Hmg/8.7.1219 |
0.561 |
|
| 1999 |
Monani UR, Lorson CL, Parsons DW, Prior TW, Androphy EJ, Burghes AHM, McPherson JD. A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2 Human Molecular Genetics. 8: 1177-1183. PMID 10369862 DOI: 10.1093/Hmg/8.7.1177 |
0.515 |
|
| 1999 |
Lorson CL, Hahnen E, Androphy EJ, Wirth B. A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy. Proceedings of the National Academy of Sciences of the United States of America. 96: 6307-11. PMID 10339583 DOI: 10.1073/Pnas.96.11.6307 |
0.579 |
|
| 1998 |
Lorson CL, Androphy EJ. The domain encoded by exon 2 of the survival motor neuron protein mediates nucleic acid binding. Human Molecular Genetics. 7: 1269-75. PMID 9668169 DOI: 10.1093/Hmg/7.8.1269 |
0.541 |
|
| 1998 |
Lorson CL, Strasswimmer J, Yao JM, Baleja JD, Hahnen E, Wirth B, Le T, Burghes AH, Androphy EJ. SMN oligomerization defect correlates with spinal muscular atrophy severity. Nature Genetics. 19: 63-6. PMID 9590291 DOI: 10.1038/Ng0598-63 |
0.526 |
|
| 1998 |
Lorson C, Pearson J, Burger L, Pintel DJ. An SP1-binding site and TATA element are sufficient to support full transactivation by proximally bound NS1 protein of minute virus of mice Virology. 240: 326-337. PMID 9454706 DOI: 10.1006/Viro.1997.8940 |
0.341 |
|
| 1997 |
Lorson C, Pintel DJ. Characterization of the minute virus of mice P38 core promoter elements Journal of Virology. 71: 6568-6575. PMID 9261378 DOI: 10.1128/Jvi.71.9.6568-6575.1997 |
0.302 |
|
| 1996 |
Lorson C, Burger LR, Mouw M, Pintel DJ. Efficient transactivation of the minute virus of mice P38 promoter requires upstream binding of NS1 Journal of Virology. 70: 834-842. PMID 8551622 DOI: 10.1128/Jvi.70.2.834-842.1996 |
0.303 |
|
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