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ArticlesPeer ReviewedFull Text
Molecular biology of amyotrophic lateral sclerosis: insights from genetics
Author / Creator:Pasinelli, Piera ; Brown, Robert H.
Publisher:London: Nature Publishing Group UK
Journal title:Nature reviews. Neuroscience, 2006-09, Vol.7 (9), p.710-723
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Repeat‐associated non‐AUG translation in C9orf72‐ALS/FTD is driven by neuronal excitation and stress
Author / Creator:Westergard, Thomas ; McAvoy, Kevin ; Russell, Katelyn ; Wen, Xinmei ; Pang, Yu ; Morris, Brandie ; Pasinelli, Piera ; Trotti, Davide ; Haeusler, Aaron
Publisher:London: Nature Publishing Group UK
Journal title:EMBO molecular medicine, 2019-02, Vol.11 (2), p.1-n/a
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A mouse model with widespread expression of the C9orf72-linked glycine–arginine dipeptide displays non-lethal ALS/FTD-like phenotypes
Author / Creator:Verdone, Brandie Morris ; Cicardi, Maria Elena ; Wen, Xinmei ; Sriramoji, Sindhu ; Russell, Katelyn ; Markandaiah, Shashirekha S. ; Jensen, Brigid K. ; Krishnamurthy, Karthik ; Haeusler, Aaron R. ; Pasinelli, Piera ; Trotti, Davide
Publisher:London: Nature Publishing Group UK
Journal title:Scientific reports, 2022-04, Vol.12 (1), p.5644-17, Article 5644
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over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1
Author / Creator:Guareschi, Stefania ; Cova, Emanuela ; Cereda, Cristina ; Ceroni, Mauro ; Donetti, Elena ; Bosco, Daryl A ; Trotti, Davide ; Pasinelli, Piera
Publisher:United States: National Academy of Sciences
Journal title:Proceedings of the National Academy of Sciences - PNAS, 2012-03, Vol.109 (13), p.5074-5079
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ArticlesPeer ReviewedFull Text
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
Author / Creator:Bosco, Daryl A ; Morfini, Gerardo ; Karabacak, N Murat ; Song, Yuyu ; Gros-Louis, Francois ; Pasinelli, Piera ; Goolsby, Holly ; Fontaine, Benjamin A ; Lemay, Nathan ; McKenna-Yasek, Diane ; Frosch, Matthew P ; Agar, Jeffrey N ; Julien, Jean-Pierre ; Brady, Scott T ; Brown, Robert H
Publisher:New York: Nature Publishing Group US
Journal title:Nature neuroscience, 2010-11, Vol.13 (11), p.1396-1403
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Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
Author / Creator:Casci, Ian ; Krishnamurthy, Karthik ; Kour, Sukhleen ; Tripathy, Vadreenath ; Ramesh, Nandini ; Anderson, Eric N. ; Marrone, Lara ; Grant, Rogan A. ; Oliver, Stacie ; Gochenaur, Lauren ; Patel, Krishani ; Sterneckert, Jared ; Gleixner, Amanda M. ; Donnelly, Christopher J. ; Ruepp, Marc-David ; Sini, Antonella M. ; Zuccaro, Emanuela ; Pennuto, Maria ; Pasinelli, Piera ; Pandey, Udai Bhan
Publisher:London: Nature Publishing Group UK
Journal title:Nature communications, 2019-12, Vol.10 (1), p.5583-20, Article 5583
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EphrinB2 knockdown in cervical spinal cord preserves diaphragm innervation in a mutant SOD1 mouse model of ALS
Author / Creator:Urban, Mark W ; Charsar, Brittany A ; Heinsinger, Nicolette M ; Markandaiah, Shashirekha S ; Sprimont, Lindsay ; Zhou, Wei ; Brown, Eric V ; Henderson, Nathan T ; Thomas, Samantha J ; Ghosh, Biswarup ; Cain, Rachel E ; Trotti, Davide ; Pasinelli, Piera ; Wright, Megan C ; Dalva, Matthew B ; Lepore, Angelo C
Publisher:England: eLife Sciences Publications Ltd
Journal title:eLife, 2024-01, Vol.12
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Synaptic dysfunction induced by glycine‐alanine dipeptides in C9orf72‐ALS/FTD is rescued by SV2 replenishment
Author / Creator:Jensen, Brigid K ; Schuldi, Martin H ; McAvoy, Kevin ; Russell, Katelyn A ; Boehringer, Ashley ; Curran, Bridget M ; Krishnamurthy, Karthik ; Wen, Xinmei ; Westergard, Thomas ; Ma, Le ; Haeusler, Aaron R ; Edbauer, Dieter ; Pasinelli, Piera ; Trotti, Davide
Publisher:London: Nature Publishing Group UK
Journal title:EMBO molecular medicine, 2020-05, Vol.12 (5), p.e10722-n/a
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ArticlesPeer ReviewedFull Text
Inhibiting drug efflux transporters improves efficacy of ALS therapeutics
Author / Creator:Jablonski, Michael R. ; Markandaiah, Shashirekha S. ; Jacob, Dena ; Meng, Ni J. ; Li, Ke ; Gennaro, Victoria ; Lepore, Angelo C. ; Trotti, Davide ; Pasinelli, Piera
Publisher:United States: John Wiley & Sons, Inc
Journal title:Annals of clinical and translational neurology, 2014-12, Vol.1 (12), p.996-1005
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ArticlesPeer ReviewedFull Text
Caspase-1 and -3 Are Sequentially Activated in Motor Neuron Death in Cu,Zn Superoxide Dismutase-Mediated Familial Amyotrophic Lateral Sclerosis
Author / Creator:Pasinelli, Piera ; Houseweart, Megan K. ; Brown, Robert H. ; Cleveland, Don W.
Publisher:United States: National Academy of Sciences of the United States of America
Journal title:Proceedings of the National Academy of Sciences - PNAS, 2000-12, Vol.97 (25), p.13901-13906
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ArticlesPeer ReviewedFull Text
Caspase-1 is Activated in Neural Cells and Tissue with Amyotrophic Lateral Sclerosis-Associated Mutations in Copper-Zinc Superoxide Dismutase
Author / Creator:Pasinelli, Piera ; Borchelt, David R. ; Houseweart, Megan K. ; Cleveland, Don W. ; Brown, Robert H.
Publisher:United States: National Academy of Sciences of the United States of America
Journal title:Proceedings of the National Academy of Sciences - PNAS, 1998-12, Vol.95 (26), p.15763-15768
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ArticlesPeer ReviewedFull Text
RNA Interference-Mediated Silencing of Mutant Superoxide Dismutase Rescues Cyclosporin A-Induced Death in Cultured Neuroblastoma Cells
Author / Creator:Maxwell, Michele M. ; Pasinelli, Piera ; Kazantsev, Aleksey G. ; Brown, Robert H. ; Housman, David E.
Publisher:United States: National Academy of Sciences
Journal title:Proceedings of the National Academy of Sciences - PNAS, 2004-03, Vol.101 (9), p.3178-3183
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ArticlesPeer ReviewedFull Text
Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity
Author / Creator:Daigle, J. Gavin ; Krishnamurthy, Karthik ; Ramesh, Nandini ; Casci, Ian ; Monaghan, John ; McAvoy, Kevin ; Godfrey, Earl W. ; Daniel, Dianne C. ; Johnson, Edward M. ; Monahan, Zachary ; Shewmaker, Frank ; Pasinelli, Piera ; Pandey, Udai Bhan
Publisher:Berlin/Heidelberg: Springer Berlin Heidelberg
Journal title:Acta neuropathologica, 2016-04, Vol.131 (4), p.605-620
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ArticlesPeer ReviewedFull Text
EphrinB2 knockdown in cervical spinal cord preserves diaphragm innervation in a mutant SOD1 mouse model of ALS
Author / Creator:Urban, Mark W ; Charsar, Brittany A ; Heinsinger, Nicolette M ; Markandaiah, Shashirekha S ; Sprimont, Lindsay ; Zhou, Wei ; Brown, Eric V ; Henderson, Nathan T ; Thomas, Samantha J ; Ghosh, Biswarup ; Cain, Rachel E ; Trotti, Davide ; Pasinelli, Piera ; Wright, Megan C ; Dalva, Matthew B ; Lepore, Angelo C
Journal title:eLife, 2024-01, Vol.12
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ArticlesPeer ReviewedFull Text
Synaptic dysfunction induced by glycine‐alanine dipeptides in C9orf72‐ ALS / FTD is rescued by SV 2 replenishment
Author / Creator:Jensen, Brigid K ; Schuldi, Martin H ; McAvoy, Kevin ; Russell, Katelyn A ; Boehringer, Ashley ; Curran, Bridget M ; Krishnamurthy, Karthik ; Wen, Xinmei ; Westergard, Thomas ; Ma, Le ; Haeusler, Aaron R ; Edbauer, Dieter ; Pasinelli, Piera ; Trotti, Davide
Journal title:EMBO molecular medicine, 2020-05, Vol.12 (5)
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Neuronal Activity-Dependent Gene Dysregulation in C9orf72 i3 Neuronal Models of ALS/FTD Pathogenesis
Author / Creator:Ghaffari, Layla T ; Welebob, Emily ; Boehringer, Ashley ; Cyliax, Kelly ; Pasinelli, Piera ; Trotti, Davide ; Haeusler, Aaron R
Publisher:Cold Spring Harbor: Cold Spring Harbor Laboratory Press
Journal title:bioRxiv, 2025-01
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Astrocytic expression of ALS-causative mutant FUS leads to TNFa-dependent neurodegeneration in vivo
Author / Creator:Jensen, Brigid K ; Mcavoy, Kevin J ; Heinsinger, Nicolette M ; Lepore, Angelo C ; Ilieva, Hristelina ; Haeusler, Aaron ; Trotti, Davide ; Pasinelli, Piera
Publisher:Cold Spring Harbor: Cold Spring Harbor Laboratory Press
Journal title:bioRxiv, 2021-11
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A mouse model with widespread expression of the C9orf72-linked glycine-arginine dipeptide displays non-lethal ALS/FTD-like phenotypes
Author / Creator:Verdone, Brandie ; Cicardi, Maria ; Wen, Xinmei ; Sriramoji, Sindhu ; Russell, Katelyn ; Shashirekha Markandaiah ; Jensen, Brigid ; Krishnamurthy, Karthik ; Haeusler, Aaron ; Pasinelli, Piera ; Trotti, Davide
Publisher:Durham: Research Square
Journal title:Scientific Reports, 2022
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KapBeta2 is a modifier of the C9orf72-linked glycine-arginine dipeptide neurotoxicity
Author / Creator:Cicardi, Maria Elena ; Kankate, Vaishnavi ; Sriramoji, Sindhu ; Krishnamurthy, Karthik ; Shashirekha Shamamandrimarkandaiah ; Verdone-Morris, Brandie ; Girdhar, Amandeep ; Nelson, Andrew T ; Rivas, Linda B ; Boehringer, Ashley ; Haeusler, Aaron ; Pasinelli, Piera ; Guo, Lin ; Trotti, Davide
Publisher:Cold Spring Harbor: Cold Spring Harbor Laboratory Press
Journal title:bioRxiv, 2022-10
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C9orf72 poly(PR) mediated neurodegeneration is associated with nucleolar stress
Author / Creator:Cicardi, Maria Elena ; Hallgren, Justin ; Darilang, Mawrie ; Krishnamurthy, Karthik ; Shashirekha Shamamandri Markandaiah ; Nelson, Andrew T ; Kankate, Vaishnavi ; Anderson, Eric ; Pasinelli, Piera ; Udai Bhan Pandey ; Eischen, Christine M ; Trotti, Davide
Publisher:Cold Spring Harbor: Cold Spring Harbor Laboratory Press
Journal title:bioRxiv, 2023-02
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Molecular biology of amyotrophic lateral sclerosis: insights from genetics

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Repeat‐associated non‐AUG translation in C9orf72‐ALS/FTD is driven by neuronal excitation and stress

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A mouse model with widespread expression of the C9orf72-linked glycine–arginine dipeptide displays non-lethal ALS/FTD-like phenotypes

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over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1

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Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

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Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization

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EphrinB2 knockdown in cervical spinal cord preserves diaphragm innervation in a mutant SOD1 mouse model of ALS

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Synaptic dysfunction induced by glycine‐alanine dipeptides in C9orf72‐ALS/FTD is rescued by SV2 replenishment

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Inhibiting drug efflux transporters improves efficacy of ALS therapeutics

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Caspase-1 and -3 Are Sequentially Activated in Motor Neuron Death in Cu,Zn Superoxide Dismutase-Mediated Familial Amyotrophic Lateral Sclerosis

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Caspase-1 is Activated in Neural Cells and Tissue with Amyotrophic Lateral Sclerosis-Associated Mutations in Copper-Zinc Superoxide Dismutase

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RNA Interference-Mediated Silencing of Mutant Superoxide Dismutase Rescues Cyclosporin A-Induced Death in Cultured Neuroblastoma Cells

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Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity

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EphrinB2 knockdown in cervical spinal cord preserves diaphragm innervation in a mutant SOD1 mouse model of ALS

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Synaptic dysfunction induced by glycine‐alanine dipeptides in C9orf72‐ ALS / FTD is rescued by SV 2 replenishment

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Neuronal Activity-Dependent Gene Dysregulation in C9orf72 i3 Neuronal Models of ALS/FTD Pathogenesis

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Astrocytic expression of ALS-causative mutant FUS leads to TNFa-dependent neurodegeneration in vivo

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A mouse model with widespread expression of the C9orf72-linked glycine-arginine dipeptide displays non-lethal ALS/FTD-like phenotypes

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KapBeta2 is a modifier of the C9orf72-linked glycine-arginine dipeptide neurotoxicity

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C9orf72 poly(PR) mediated neurodegeneration is associated with nucleolar stress

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