Citations (123)
Keep up to date with the latest research on this topic with citation updates for this article.
Read on this site (4)
Vladimir N. Uversky. (2017) The roles of intrinsic disorder-based liquid-liquid phase transitions in the “Dr. Jekyll–Mr. Hyde” behavior of proteins involved in amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Autophagy 13:12, pages 2115-2162.
Read now
Read now
Leslie I Grad & Neil R Cashman. (2014) Prion-like activity of Cu/Zn superoxide dismutase. Prion 8:1, pages 33-41.
Read now
Read now
Lewis P. Rowland. (2009) T.L. Bunina, Asao Hirano, and the post mortem cellular diagnosis of amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis 10:2, pages 74-78.
Read now
Read now
Lucie I Bruijn & Merit Cudkowicz. (2006) Therapeutic targets for amyotrophic lateral sclerosis: current treatments and prospects for more effective therapies. Expert Review of Neurotherapeutics 6:3, pages 417-428.
Read now
Read now
Articles from other publishers (119)
Abhirami K. Iyer, Kathleen M. Schoch, Anthony Verbeck, Grant Galasso, Hao Chen, Sarah Smith, Anna Oldenborg, Timothy M. Miller, Celeste M. Karch & Azad Bonni. (2023) Targeted ASO-mediated Atp1a2 knockdown in astrocytes reduces SOD1 aggregation and accelerates disease onset in mutant SOD1 mice. PLOS ONE 18:11, pages e0294731.
Crossref
Crossref
Sara S. Ribeiro, David Gnutt, Salome Azoulay-Ginsburg, Zamira Fetahaj, Ella Spurlock, Felix Lindner, Damon Kuz, Yfat Cohen-Erez, Hanna Rapaport, Adrian Israelson, Arie-lev Gruzman & Simon Ebbinghaus. (2023) Intracellular spatially-targeted chemical chaperones increase native state stability of mutant SOD1 barrel. Biological Chemistry 404:10, pages 909-930.
Crossref
Crossref
Jacob I. Ayers, Guilian Xu, Qing Lu, Kristy Dillon, Susan Fromholt & David R. Borchelt. (2023) Multiple Factors Influence the Incubation Period of ALS Prion-like Transmission in SOD1 Transgenic Mice. Viruses 15:9, pages 1819.
Crossref
Crossref
Olga-Maria Iova, Gheorghe-Eduard Marin, Izabella Lazar, Ioana Stanescu, Gabriela Dogaru, Cristina Ariadna Nicula & Adriana Elena Bulboacă. (2023) Nitric Oxide/Nitric Oxide Synthase System in the Pathogenesis of Neurodegenerative Disorders—An Overview. Antioxidants 12:3, pages 753.
Crossref
Crossref
Ulrika Nordström, Lisa Lang, Elaheh Ekhtiari Bidhendi, Per Zetterström, Mikael Oliveberg, Jens Danielsson, Peter M. Andersen & Stefan L. Marklund. (2022)
Mutant
SOD1
aggregates formed in vitro and in cultured cells are polymorphic and differ from those arising in the
CNS
. Journal of Neurochemistry 164:1, pages 77-93.
Crossref
Crossref
Karin M. Forsberg, Karin S. Graffmo, Erica Stenvall, Naima Tabikh, Stefan L. Marklund, Thomas Brännström & Peter M. Andersen. (2022) Widespread CNS pathology in amyotrophic lateral sclerosis homozygous for the D90A SOD1 mutation. Acta Neuropathologica 145:1, pages 13-28.
Crossref
Crossref
Julien H Park, Ulrika Nordström, Konstantinos Tsiakas, Isil Keskin, Christiane Elpers, Manoj Mannil, Raoul Heller, Melinda Nolan, Salam Alburaiky, Per Zetterström, Maja Hempel, Ulrike Schara-Schmidt, Saskia Biskup, Petra Steinacker, Markus Otto, Jochen Weishaupt, Andreas Hahn, René Santer, Thorsten Marquardt, Stefan L Marklund & Peter M Andersen. (2023) The motor system is exceptionally vulnerable to absence of the ubiquitously expressed superoxide dismutase-1. Brain Communications 5:1.
Crossref
Crossref
Juan José Casañas & María Luz Montesinos. (2022) Proteomic characterization of spinal cord synaptoneurosomes from Tg-SOD1/G93A mice supports a role for MNK1 and local translation in the early stages of amyotrophic lateral sclerosis. Molecular and Cellular Neuroscience 123, pages 103792.
Crossref
Crossref
Benjamin G Trist, Sian Genoud, Stéphane Roudeau, Alexander Rookyard, Amr Abdeen, Veronica Cottam, Dominic J Hare, Melanie White, Jens Altvater, Jennifer A Fifita, Alison Hogan, Natalie Grima, Ian P Blair, Kai Kysenius, Peter J Crouch, Asuncion Carmona, Yann Rufin, Stéphane Claverol, Stijn Van Malderen, Gerald Falkenberg, David J Paterson, Bradley Smith, Claire Troakes, Caroline Vance, Christopher E Shaw, Safa Al-Sarraj, Stuart Cordwell, Glenda Halliday, Richard Ortega & Kay L Double. (2022) Altered SOD1 maturation and post-translational modification in amyotrophic lateral sclerosis spinal cord. Brain 145:9, pages 3108-3130.
Crossref
Crossref
Harmeen K. Deol, Helen R. Broom, Bruna Siebeneichler, Brenda Lee, Zoya Leonenko & Elizabeth M. Meiering. (2022) Immature ALS-associated mutant superoxide dismutases form variable aggregate structures through distinct oligomerization processes. Biophysical Chemistry 288, pages 106844.
Crossref
Crossref
Shima Famil Samavati, Maryam Nikkhah, Akram Eidi & Reza Khodarahmi. (2021) Study on the effects of various incubation conditions on aggregation of SOD1 variants: disulfide bond reduction and demetallation synergistically promote generation of non-amyloid amorphous aggregates from SOD1 mutants. Journal of the Iranian Chemical Society 19:5, pages 1755-1771.
Crossref
Crossref
René Günther, Arun Pal, Chloe Williams, Vitaly L. Zimyanin, Maria Liehr, Cläre von Neubeck, Mechthild Krause, Mrudula G. Parab, Susanne Petri, Norman Kalmbach, Stefan L. Marklund, Jared Sterneckert, Peter Munch Andersen, Florian Wegner, Jonathan D. Gilthorpe & Andreas Hermann. (2022) Alteration of Mitochondrial Integrity as Upstream Event in the Pathophysiology of SOD1-ALS. Cells 11:7, pages 1246.
Crossref
Crossref
Oluwabusuyi Rachael Folarin, Funmilayo E Olopade & James Olukayode Olopade. (2021) Essential Metals in the Brain and the Application of Laser Ablation-Inductively Coupled Plasma-Mass Spectrometry for their Detection. Nigerian Journal of Physiological Sciences 36:2, pages 123-147.
Crossref
Crossref
Isil Keskin, Elaheh Ekhtiari Bidhendi, Matthew Marklund, Peter M. Andersen, Thomas Brännström, Stefan L. Marklund & Ulrika Nordström. (2021) Peripheral administration of SOD1 aggregates does not transmit pathogenic aggregation to the CNS of SOD1 transgenic mice. Acta Neuropathologica Communications 9:1.
Crossref
Crossref
François Muratet, Elisa Teyssou, Aude Chiot, Séverine Boillée, Christian S Lobsiger, Delphine Bohl, Beata Gyorgy, Justine Guegan, Yannick Marie, Maria del Mar Amador, Francois Salachas, Vincent Meininger, Emilien Bernard, Jean-Christophe Antoine, Jean-Philippe Camdessanché, William Camu, Cécile Cazeneuve, Anne-Laure Fauret-Amsellem, Eric Leguern, Kevin Mouzat, Claire Guissart, Serge Lumbroso, Philippe Corcia, Patrick Vourc'h, Aude-Marie Grapperon, Shahram Attarian, Annie Verschueren, Danielle Seilhean & Stéphanie Millecamps. (2021)
Impact of a frequent nearsplice
SOD1
variant in amyotrophic lateral sclerosis: optimising
SOD1
genetic screening for gene therapy opportunities
. Journal of Neurology, Neurosurgery & Psychiatry 92:9, pages 942-949.
Crossref
Crossref
Christen G. Chisholm, Justin J. Yerbury & Luke McAlary. 2021. Spectrums of Amyotrophic Lateral Sclerosis. Spectrums of Amyotrophic Lateral Sclerosis
105
121
.
Benjamin G. Trist, James B. Hilton, Dominic J. Hare, Peter J. Crouch & Kay L. Double. (2020) Superoxide Dismutase 1 in Health and Disease: How a Frontline Antioxidant Becomes Neurotoxic. Angewandte Chemie International Edition 60:17, pages 9215-9246.
Crossref
Crossref
Benjamin G. Trist, James B. Hilton, Dominic J. Hare, Peter J. Crouch & Kay L. Double. (2020) Superoxide Dismutase 1 in Health and Disease: How a Frontline Antioxidant Becomes Neurotoxic. Angewandte Chemie 133:17, pages 9299-9330.
Crossref
Crossref
Luke McAlary, Yee Lian Chew, Jeremy Stephen Lum, Nicholas John Geraghty, Justin John Yerbury & Neil R. Cashman. (2020) Amyotrophic Lateral Sclerosis: Proteins, Proteostasis, Prions, and Promises. Frontiers in Cellular Neuroscience 14.
Crossref
Crossref
Shima Famil Samavati, Maryam Nikkhah, Akram Eidi & Reza Khodarahmi. (2020) Reduced thermodynamic stability as prerequisite for aggregation of SOD1 mutants: a path through the reduction in intramolecular disulfide bonds. Journal of the Iranian Chemical Society 17:8, pages 2053-2071.
Crossref
Crossref
Dharma Rao Tompa & Saraboji Kadhirvel. (2020) Changes in hydrophobicity mainly promotes the aggregation tendency of ALS associated SOD1 mutants. International Journal of Biological Macromolecules 145, pages 904-913.
Crossref
Crossref
L. McAlary, J.J. Yerbury & N.R. Cashman. 2020. Prions and Neurodegenerative Diseases. Prions and Neurodegenerative Diseases
261
296
.
Cameron Wells, Samuel E. Brennan, Matt Keon & Nitin K. Saksena. (2019) Prionoid Proteins in the Pathogenesis of Neurodegenerative Diseases. Frontiers in Molecular Neuroscience 12.
Crossref
Crossref
Luke McAlary, Steven S. Plotkin, Justin J. Yerbury & Neil R. Cashman. (2019) Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis. Frontiers in Molecular Neuroscience 12.
Crossref
Crossref
Vijaya Kumar Hinge, Nikolay Blinov, Dipankar Roy, David S. Wishart & Andriy Kovalenko. (2019) The role of hydration effects in 5-fluorouridine binding to SOD1: insight from a new 3D-RISM-KH based protocol for including structural water in docking simulations. Journal of Computer-Aided Molecular Design 33:10, pages 913-926.
Crossref
Crossref
Cassandra Terry & Jonathan D. F. Wadsworth. (2019) Recent Advances in Understanding Mammalian Prion Structure: A Mini Review. Frontiers in Molecular Neuroscience 12.
Crossref
Crossref
Cindy Gill, James P. Phelan, Theo Hatzipetros, Joshua D. Kidd, Valerie R. Tassinari, Beth Levine, Monica Z. Wang, Andrew Moreno, Kenneth Thompson, Marcel Maier, Jan Grimm, Alan Gill & Fernando G. Vieira. (2019) SOD1-positive aggregate accumulation in the CNS predicts slower disease progression and increased longevity in a mutant SOD1 mouse model of ALS. Scientific Reports 9:1.
Crossref
Crossref
Alida Spalloni, Michele Nutini & Patrizia Longone. 2019. Pathology, Prevention and Therapeutics of Neurodegenerative Disease. Pathology, Prevention and Therapeutics of Neurodegenerative Disease
61
71
.
E. Srinivasan & R. Rajasekaran. (2018) Quantum chemical and molecular mechanics studies on the assessment of interactions between resveratrol and mutant SOD1 (G93A) protein. Journal of Computer-Aided Molecular Design 32:12, pages 1347-1361.
Crossref
Crossref
Elaheh Ekhtiari Bidhendi, Johan Bergh, Per Zetterström, Karin Forsberg, Bente Pakkenberg, Peter M. Andersen, Stefan L. Marklund & Thomas Brännström. (2018) Mutant superoxide dismutase aggregates from human spinal cord transmit amyotrophic lateral sclerosis. Acta Neuropathologica 136:6, pages 939-953.
Crossref
Crossref
Emiliano Trias, Luis Barbeito & Koji Yamanaka. (2018) Phenotypic heterogeneity of astrocytes in motor neuron disease. Clinical and Experimental Neuroimmunology 9:4, pages 225-234.
Crossref
Crossref
Lucas S. Dantas, Adriano B. Chaves-Filho, Fernando R. Coelho, Thiago C. Genaro-Mattos, Keri A. Tallman, Ned A. Porter, Ohara Augusto & Sayuri Miyamoto. (2018) Cholesterol secosterol aldehyde adduction and aggregation of Cu,Zn-superoxide dismutase: Potential implications in ALS. Redox Biology 19, pages 105-115.
Crossref
Crossref
E. Srinivasan & R. Rajasekaran. (2018) Comparative binding of kaempferol and kaempferide on inhibiting the aggregate formation of mutant (G85R) SOD1 protein in familial amyotrophic lateral sclerosis: A quantum chemical and molecular mechanics study. BioFactors 44:5, pages 431-442.
Crossref
Crossref
Robert H Scannevin. (2018) Therapeutic strategies for targeting neurodegenerative protein misfolding disorders. Current Opinion in Chemical Biology 44, pages 66-74.
Crossref
Crossref
E. Srinivasan & R. Rajasekaran. (2017) Cysteine to Serine Conversion at 111th Position Renders the Disaggregation and Retains the Stabilization of Detrimental SOD1 A4V Mutant Against Amyotrophic Lateral Sclerosis in Human—A Discrete Molecular Dynamics Study. Cell Biochemistry and Biophysics 76:1-2, pages 231-241.
Crossref
Crossref
Ramu Manjula, Gareth S. A. Wright, Richard W. Strange & Balasundaram Padmanabhan. (2018)
Assessment of ligand binding at a site relevant to
SOD
1 oxidation and aggregation
. FEBS Letters 592:10, pages 1725-1737.
Crossref
Crossref
Veronica Hirsch-Reinshagen, Cyril Pottier, Alexandra M. Nicholson, Matt Baker, Ging-Yuek R. Hsiung, Charles Krieger, Pheth Sengdy, Kevin B. Boylan, Dennis W. Dickson, Marsel Mesulam, Sandra Weintraub, Eileen Bigio, Lorne Zinman, Julia Keith, Ekaterina Rogaeva, Sasha A. Zivkovic, David Lacomis, J. Paul Taylor, Rosa Rademakers & Ian R. A. Mackenzie. (2017) Clinical and neuropathological features of ALS/FTD with TIA1 mutations. Acta Neuropathologica Communications 5:1.
Crossref
Crossref
Nandini Ramesh & Udai Bhan Pandey. (2017) Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand. Frontiers in Molecular Neuroscience 10.
Crossref
Crossref
Leslie I. Grad, Guy A. Rouleau, John Ravits & Neil R. Cashman. (2017) Clinical Spectrum of Amyotrophic Lateral Sclerosis (ALS). Cold Spring Harbor Perspectives in Medicine 7:8, pages a024117.
Crossref
Crossref
Vincent Picher-Martel, Paul N. Valdmanis, Peter V. Gould, Jean-Pierre Julien & Nicolas Dupré. (2016) From animal models to human disease: a genetic approach for personalized medicine in ALS. Acta Neuropathologica Communications 4:1.
Crossref
Crossref
Sarah Pickles, Sabrina Semmler, Helen R. Broom, Laurie Destroismaisons, Laurine Legroux, Nathalie Arbour, Elizabeth Meiering, Neil R. Cashman & Christine Vande Velde. (2016) ALS-linked misfolded SOD1 species have divergent impacts on mitochondria. Acta Neuropathologica Communications 4:1.
Crossref
Crossref
Eiichi Tokuda, Thomas Brännström, Peter M. Andersen & Stefan L. Marklund. (2016) Low autophagy capacity implicated in motor system vulnerability to mutant superoxide dismutase. Acta Neuropathologica Communications 4:1.
Crossref
Crossref
Shinsuke Kato, Masako Kato, Teruo Kusano & Takeshi Nishino. (2016) New Strategy That Delays Progression of Amyotrophic Lateral Sclerosis in G1H-G93A Transgenic Mice: Oral Administration of Xanthine Oxidoreductase Inhibitors That Are Not Substrates for the Purine Salvage Pathway. Journal of Neuropathology & Experimental Neurology 75:12, pages 1124-1144.
Crossref
Crossref
Ashok SekharJessica A. O. RumfeldtHelen R. BroomColleen M. DoyleRyan E. SoberingElizabeth M. MeieringLewis E. Kay. (2016) Probing the free energy landscapes of ALS disease mutants of SOD1 by NMR spectroscopy. Proceedings of the National Academy of Sciences 113:45.
Crossref
Crossref
Roberta Cascella, Claudia Capitini, Giulia Fani, Christopher M. Dobson, Cristina Cecchi & Fabrizio Chiti. (2016) Quantification of the Relative Contributions of Loss-of-function and Gain-of-function Mechanisms in TAR DNA-binding Protein 43 (TDP-43) Proteinopathies. Journal of Biological Chemistry 291:37, pages 19437-19448.
Crossref
Crossref
Glenn Dallérac & Nathalie Rouach. (2016) Astrocytes as new targets to improve cognitive functions. Progress in Neurobiology 144, pages 48-67.
Crossref
Crossref
Elaheh Ekhtiari Bidhendi, Johan Bergh, Per Zetterström, Peter M. Andersen, Stefan L. Marklund & Thomas Brännström. (2016) Two superoxide dismutase prion strains transmit amyotrophic lateral sclerosis–like disease. Journal of Clinical Investigation 126:6, pages 2249-2253.
Crossref
Crossref
Drazen Petrov, Xavier Daura & Bojan Zagrovic. (2016) Effect of Oxidative Damage on the Stability and Dimerization of Superoxide Dismutase 1. Biophysical Journal 110:7, pages 1499-1509.
Crossref
Crossref
Isil Keskin, Elin Forsgren, Dale J. Lange, Markus Weber, Anna Birve, Matthis Synofzik, Jonathan D. Gilthorpe, Peter M. Andersen & Stefan L. Marklund. (2016) Effects of Cellular Pathway Disturbances on Misfolded Superoxide Dismutase-1 in Fibroblasts Derived from ALS Patients. PLOS ONE 11:2, pages e0150133.
Crossref
Crossref
Yoshiaki Furukawa, Itsuki Anzai, Shuji Akiyama, Mizue Imai, Fatima Joy C. Cruz, Tomohide Saio, Kenichi Nagasawa, Takao Nomura & Koichiro Ishimori. (2016) Conformational Disorder of the Most Immature Cu, Zn-Superoxide Dismutase Leading to Amyotrophic Lateral Sclerosis. Journal of Biological Chemistry 291:8, pages 4144-4155.
Crossref
Crossref
Helen R. Broom, Kenrick A. Vassall, Jessica A. O. Rumfeldt, Colleen M. Doyle, Ming Sze Tong, Julia M. Bonner & Elizabeth M. Meiering. (2016) Combined Isothermal Titration and Differential Scanning Calorimetry Define Three-State Thermodynamics of fALS-Associated Mutant Apo SOD1 Dimers and an Increased Population of Folded Monomer. Biochemistry 55:3, pages 519-533.
Crossref
Crossref
Masako Kato, Shinsuke Kato, Kiyota Kato & Kazuhiko Hayashi. (2016) Contribution of CD3 <i>ε</i> Epitope and Oxidative Type of Copper-Zinc Superoxide Dismutase to the Degeneration Processes of Cerebellar Purkinje Cells in Patients with Multiple System Atrophy-Cerebellar Type (MSA-C: Olivopontocerebellar Atrophy, OPCA): An Immunohistochemical Study. World Journal of Neuroscience 06:04, pages 310-324.
Crossref
Crossref
Arnaud Francois, Julie Verite, Agnès Rioux Bilan, Thierry Janet, Frédéric Calon, Bernard Fauconneau, Marc Paccalin & Guylène Page. 2016. Molecules to Medicine with mTOR. Molecules to Medicine with mTOR
85
104
.
Helen R. Broom, Jessica A. O. Rumfeldt, Kenrick A. Vassall & Elizabeth M. Meiering. (2015)
Destabilization of the dimer interface is a common consequence of diverse
ALS
‐associated mutations in metal free
SOD1
. Protein Science 24:12, pages 2081-2089.
Crossref
Crossref
Edoardo Del Poggetto, Fabrizio Chiti & Francesco Bemporad. (2015) The Folding process of Human Profilin-1, a novel protein associated with familial amyotrophic lateral sclerosis. Scientific Reports 5:1.
Crossref
Crossref
Leslie I. Grad, Sarah M. Fernando & Neil R. Cashman. (2015) From molecule to molecule and cell to cell: Prion-like mechanisms in amyotrophic lateral sclerosis. Neurobiology of Disease 77, pages 257-265.
Crossref
Crossref
Shynrye Lee & Hyung-Jun Kim. (2015) Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?. Experimental Neurobiology 24:1, pages 1-7.
Crossref
Crossref
Huihui Han, Wanyi Wei, Weisong Duan, Yansu Guo, Yi Li, Jie Wang, Yue Bi & Chunyan Li. (2014) Autophagy-linked FYVE protein (Alfy) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS). In Vitro Cellular & Developmental Biology - Animal 51:3, pages 249-263.
Crossref
Crossref
Mariko Ogawa & Yoshiaki Furukawa. (2014) A seeded propagation of Cu, Zn-superoxide dismutase aggregates in amyotrophic lateral sclerosis. Frontiers in Cellular Neuroscience 8.
Crossref
Crossref
Joana Cristóvão, Sónia Leal, Isabel Cardoso & Cláudio Gomes. (2013) Small Molecules Present in the Cerebrospinal Fluid Metabolome Influence Superoxide Dismutase 1 Aggregation. International Journal of Molecular Sciences 14:9, pages 19128-19145.
Crossref
Crossref
Sónia S. Leal, Isabel Cardoso, Joan S. Valentine & Cláudio M. Gomes. (2013) Calcium Ions Promote Superoxide Dismutase 1 (SOD1) Aggregation into Non-fibrillar Amyloid. Journal of Biological Chemistry 288:35, pages 25219-25228.
Crossref
Crossref
Vikram Khipple Mulligan & Avijit Chakrabartty. (2013) Protein misfolding in the late-onset neurodegenerative diseases: Common themes and the unique case of amyotrophic lateral sclerosis. Proteins: Structure, Function, and Bioinformatics 81:8, pages 1285-1303.
Crossref
Crossref
Matthew Thomas, Javier Alegre-Abarrategui & Richard Wade-Martins. (2013) RNA dysfunction and aggrephagy at the centre of an amyotrophic lateral sclerosis/frontotemporal dementia disease continuum. Brain 136:5, pages 1345-1360.
Crossref
Crossref
Gareth S.A. Wright, Svetlana V. Antonyuk, Neil M. Kershaw, Richard W. Strange & S Samar Hasnain. (2013) Ligand binding and aggregation of pathogenic SOD1. Nature Communications 4:1.
Crossref
Crossref
K. S. Graffmo, K. Forsberg, J. Bergh, A. Birve, P. Zetterstrom, P. M. Andersen, S. L. Marklund & T. Brannstrom. (2012) Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis. Human Molecular Genetics 22:1, pages 51-60.
Crossref
Crossref
Vikram Khipple Mulligan, Aaron Kerman, Rob C. Laister, Priya Roy Sharda, Pharhad Eli Arslan & Avijit Chakrabartty. (2012) Early Steps in Oxidation-Induced SOD1 Misfolding: Implications for Non-Amyloid Protein Aggregation in Familial ALS. Journal of Molecular Biology 421:4-5, pages 631-652.
Crossref
Crossref
Gerald Münch, Bernadette Westcott, Teresita Menini & Alejandro Gugliucci. (2010) Advanced glycation endproducts and their pathogenic roles in neurological disorders. Amino Acids 42:4, pages 1221-1236.
Crossref
Crossref
Aaron Kerman & Avijit Chakrabartty. 2012. Non-fibrillar Amyloidogenic Protein Assemblies - Common Cytotoxins Underlying Degenerative Diseases. Non-fibrillar Amyloidogenic Protein Assemblies - Common Cytotoxins Underlying Degenerative Diseases
257
288
.
Inhee Choi, Hyeon Don Song, Suseung Lee, Young In Yang, Joo Hyun Nam, Sung Joon Kim, Jung-Joon Sung, Taewook Kang & Jongheop Yi. (2011) Direct Observation of Defects and Increased Ion Permeability of a Membrane Induced by Structurally Disordered Cu/Zn-Superoxide Dismutase Aggregates. PLoS ONE 6:12, pages e28982.
Crossref
Crossref
Guijie Ren, Zhongcai Ma, Maria Hui, Lili C Kudo, Koon-Sea Hui & Stanislav L Karsten. (2011) Cu, Zn-superoxide dismutase 1 (SOD1) is a novel target of Puromycin-sensitive aminopeptidase (PSA/NPEPPS): PSA/NPEPPS is a possible modifier of amyotrophic lateral sclerosis. Molecular Neurodegeneration 6:1.
Crossref
Crossref
Akemi Ido, Hidenao Fukuyama & Makoto Urushitani. (2011) Protein Misdirection Inside and Outside Motor Neurons in Amyotrophic Lateral Sclerosis (ALS): A Possible Clue for Therapeutic Strategies. International Journal of Molecular Sciences 12:10, pages 6980-7003.
Crossref
Crossref
Leslie I. GradWill C. GuestAnat YanaiEdward PokrishevskyMegan A. O'NeillEbrima GibbsValentyna SemenchenkoMasoud YousefiDavid S. Wishart, Steven S. Plotkin & Neil R. Cashman. (2011) Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proceedings of the National Academy of Sciences 108:39, pages 16398-16403.
Crossref
Crossref
Mehdi Hossaini, Sebastian Cardona Cano, Vera van Dis, Elize D. Haasdijk, Casper C. Hoogenraad, Jan C. Holstege & Dick Jaarsma. (2011) Spinal Inhibitory Interneuron Pathology Follows Motor Neuron Degeneration Independent of Glial Mutant Superoxide Dismutase 1 Expression in SOD1-ALS Mice. Journal of Neuropathology & Experimental Neurology 70:8, pages 662-677.
Crossref
Crossref
Per Zetterström, Peter M. Andersen, Thomas Brännström & Stefan L. Marklund. (2011) Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patients. Journal of Neurochemistry 117:1, pages 91-99.
Crossref
Crossref
Kenrick A. VassallHelen R. StubbsHeather A. PrimmerMing Sze TongSarah M. SullivanRyan SoberingSaipraveen Srinivasan, Lee-Ann K. Briere, Stanley D. Dunn, Wilfredo Colón & Elizabeth M. Meiering. (2011) Decreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS. Proceedings of the National Academy of Sciences 108:6, pages 2210-2215.
Crossref
Crossref
Wendy W. Yang, Richard L. Sidman, Tatyana V. Taksir, Christopher M. Treleaven, Jonathan A. Fidler, Seng H. Cheng, James C. Dodge & Lamya S. Shihabuddin. (2011) Relationship between neuropathology and disease progression in the SOD1G93A ALS mouse. Experimental Neurology 227:2, pages 287-295.
Crossref
Crossref
Yansu Guo, Chunyan Li, Dongxia Wu, Shuyu Wu, Cheng Yang, Yaling Liu, Hongran Wu & Zhongyao Li. (2010) Ultrastructural diversity of inclusions and aggregations in the lumbar spinal cord of SOD1-G93A transgenic mice. Brain Research 1353, pages 234-244.
Crossref
Crossref
Karin Forsberg, P. Andreas Jonsson, Peter M. Andersen, Daniel Bergemalm, Karin S. Graffmo, Magnus Hultdin, Johan Jacobsson, Roland Rosquist, Stefan L. Marklund & Thomas Brännström. (2010) Novel Antibodies Reveal Inclusions Containing Non-Native SOD1 in Sporadic ALS Patients. PLoS ONE 5:7, pages e11552.
Crossref
Crossref
Y. Kawamoto, H. Ito, Y. Kobayashi, Y. Suzuki, I. Akiguchi, H. Fujimura, S. Sakoda, H. Kusaka, A. Hirano & R. Takahashi. (2010) HtrA2/Omi-immunoreactive intraneuronal inclusions in the anterior horn of patients with sporadic and Cu/Zn superoxide dismutase (SOD1) mutant amyotrophic lateral sclerosis. Neuropathology and Applied Neurobiology 36:4, pages 331-344.
Crossref
Crossref
Aaron Kerman, Hsueh-Ning Liu, Sidney Croul, Juan Bilbao, Ekaterina Rogaeva, Lorne Zinman, Janice Robertson & Avijit Chakrabartty. (2010) Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form. Acta Neuropathologica 119:3, pages 335-344.
Crossref
Crossref
Florencia Palacios, Germán Cota, Sofía Horjales, Analía Lima, Julio Battistoni, José Sotelo‐Silveira & Mónica Marín. (2010)
An antibody‐based affinity chromatography tool to assess Cu, Zn superoxide dismutase (SOD) G93A structural complexity
in vivo
. Biotechnology Journal 5:3, pages 328-334.
Crossref
Crossref
Satomi Maekawa, P. Nigel Leigh, Andrew King, Edith Jones, John C. Steele, Istvan Bodi, Christopher E. Shaw, Tibor Hortobagyi & Safa Al-Sarraj. (2009) 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. Neuropathology 29:6, pages 672-683.
Crossref
Crossref
Madhuri ChattopadhyayJoan Selverstone Valentine. (2009) Aggregation of Copper–Zinc Superoxide Dismutase in Familial and Sporadic ALS. Antioxidants & Redox Signaling 11:7, pages 1603-1614.
Crossref
Crossref
Zeynep A. Oztug Durer, Jeffrey A. Cohlberg, Phong Dinh, Shelby Padua, Krista Ehrenclou, Sean Downes, James K. Tan, Yoko Nakano, Christopher J. Bowman, Jessica L. Hoskins, Chuhee Kwon, Andrew Z. Mason, Jorge A. Rodriguez, Peter A. Doucette, Bryan F. Shaw & Joan Selverstone Valentine. (2009) Loss of Metal Ions, Disulfide Reduction and Mutations Related to Familial ALS Promote Formation of Amyloid-Like Aggregates from Superoxide Dismutase. PLoS ONE 4:3, pages e5004.
Crossref
Crossref
Hisae Sumi, Shinsuke Kato, Yuko Mochimaru, Harutoshi Fujimura, Masaki Etoh & Saburo Sakoda. (2009) Nuclear TAR DNA Binding Protein 43 Expression in Spinal Cord Neurons Correlates With the Clinical Course in Amyotrophic Lateral Sclerosis. Journal of Neuropathology & Experimental Neurology 68:1, pages 37-47.
Crossref
Crossref
Fumiaki Mori, Kunikazu Tanji, Hai-Xin Zhang, Yasushi Nishihira, Chun-Feng Tan, Hitoshi Takahashi & Koichi Wakabayashi. (2008) Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia. Acta Neuropathologica 116:2, pages 193-203.
Crossref
Crossref
Makoto Urushitani, Samer Abou Ezzi, Akinori Matsuo, Ikuo Tooyama & Jean-Pierre Julien. (2008) The endoplasmic reticulum‐Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS. The FASEB Journal 22:7, pages 2476-2487.
Crossref
Crossref
P. Andreas Jonsson, Daniel Bergemalm, Peter M. Andersen, Ole Gredal, Thomas Brännström & Stefan L. Marklund. (2008) Inclusions of amyotrophic lateral sclerosis-linked superoxide dismutase in ventral horns, liver, and kidney. Annals of Neurology 63:5, pages 671-675.
Crossref
Crossref
Dick Jaarsma, Eva Teuling, Elize D. Haasdijk, Chris I. De Zeeuw & Casper C. Hoogenraad. (2008) Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induce Amyotrophic Lateral Sclerosis in Transgenic Mice. The Journal of Neuroscience 28:9, pages 2075-2088.
Crossref
Crossref
Shinsuke Kato. (2007) Amyotrophic lateral sclerosis models and human neuropathology: similarities and differences. Acta Neuropathologica 115:1, pages 97-114.
Crossref
Crossref
Satoru Yamagishi, Yoshihisa Koyama, Taiichi Katayama, Manabu Taniguchi, Junichi Hitomi, Masaaki Kato, Masashi Aoki, Yasuto Itoyama, Shinsuke Kato & Masaya Tohyama. (2007) An In Vitro Model for Lewy Body-Like Hyaline Inclusion/Astrocytic Hyaline Inclusion: Induction by ER Stress with an ALS-Linked SOD1 Mutation. PLoS ONE 2:10, pages e1030.
Crossref
Crossref
Hirofumi Yamashita, Jun Kawamata, Katsuya Okawa, Rie Kanki, Tomoki Nakamizo, Takumi Hatayama, Koji Yamanaka, Ryosuke Takahashi & Shun Shimohama. (2007) Heat-shock protein 105 interacts with and suppresses aggregation of mutant Cu/Zn superoxide dismutase: clues to a possible strategy for treating ALS. Journal of Neurochemistry 102:5, pages 1497-1505.
Crossref
Crossref
Nobutoshi Morimoto, Makiko Nagai, Yasuyuki Ohta, Kazunori Miyazaki, Tomoko Kurata, Mizuki Morimoto, Tetsuro Murakami, Yasushi Takehisa, Yoshio Ikeda, Tatsushi Kamiya & Koji Abe. (2007) Increased autophagy in transgenic mice with a G93A mutant SOD1 gene. Brain Research 1167, pages 112-117.
Crossref
Crossref
Markus Wiksten, Antti Väänänen & Päivi Liesi. (2007) Selective overexpression of γ1 laminin in astrocytes in amyotrophic lateral sclerosis indicates an involvement in ALS pathology. Journal of Neuroscience Research 85:9, pages 2045-2058.
Crossref
Crossref
Ian R. A. Mackenzie, Eileen H. Bigio, Paul G. Ince, Felix Geser, Manuela Neumann, Nigel J. Cairns, Linda K. Kwong, Mark S. Forman, John Ravits, Heather Stewart, Andrew Eisen, Leo McClusky, Hans A. Kretzschmar, Camelia M. Monoranu, J. Robin Highley, Janine Kirby, Teepu Siddique, Pamela J. Shaw, Virginia M‐Y. Lee & John Q. Trojanowski. (2007)
Pathological TDP‐43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with
SOD1
mutations
. Annals of Neurology 61:5, pages 427-434.
Crossref
Crossref
Asao Hirano & Reika Wate. (2007) Diagnostic clues and more from photographs. Neuropathology 27:1, pages 1-9.
Crossref
Crossref
Ashley Reynolds, Chad Laurie, R. Lee Mosley & Howard E. Gendelman. 2007. Neuroinflammation in Neuronal Death and Repair. Neuroinflammation in Neuronal Death and Repair
297
325
.
Tomohiro Kabuta, Yasuyuki Suzuki & Keiji Wada. (2006) Degradation of Amyotrophic Lateral Sclerosis-linked Mutant Cu,Zn-Superoxide Dismutase Proteins by Macroautophagy and the Proteasome*. Journal of Biological Chemistry 281:41, pages 30524-30533.
Crossref
Crossref
Gilmore N. O'Neill, R. Gilberto Gonzalez, Didier P. Cros, Robert H. Ackerman, Robert H. BrownJr.Jr. & Anat Stemmer-Rachamimov. (2006) Case 22-2006. New England Journal of Medicine 355:3, pages 296-304.
Crossref
Crossref
Hisae Sumi, Seiichi Nagano, Harutoshi Fujimura, Shinsuke Kato & Saburo Sakoda. (2006) Inverse correlation between the formation of mitochondria-derived vacuoles and Lewy-body-like hyaline inclusions in G93A superoxide-dismutase-transgenic mice. Acta Neuropathologica 112:1, pages 52-63.
Crossref
Crossref
Jennifer L. Wacker & Paul J. Muchowski. 2006. Protein Misfolding, Aggregation, and Conformational Diseases. Protein Misfolding, Aggregation, and Conformational Diseases
137
164
.
Masashi Aoki, Shinsuke Kato, Makiko Nagai & Yasuto Itoyama. (2005) Development of a rat model of amyotrophic lateral sclerosis expressing a human SOD1 transgene. Neuropathology 25:4, pages 365-370.
Crossref
Crossref
Gen Matsumoto, Aleksandar Stojanovic, Carina I. Holmberg, Soojin Kim & Richard I. Morimoto. (2005) Structural properties and neuronal toxicity of amyotrophic lateral sclerosis–associated Cu/Zn superoxide dismutase 1 aggregates. The Journal of Cell Biology 171:1, pages 75-85.
Crossref
Crossref
Jinbin Zhai, Hong Lin, Rafaela Canete-Soler & William W. Schlaepfer. (2005) HoxB2 binds mutant SOD1 and is altered in transgenic model of ALS. Human Molecular Genetics 14:18, pages 2629-2640.
Crossref
Crossref
Joungil Choi, Howard D. Rees, Susan T. Weintraub, Allan I. Levey, Lih-Shen Chin & Lian Li. (2005) Oxidative Modifications and Aggregation of Cu,Zn-Superoxide Dismutase Associated with Alzheimer and Parkinson Diseases. Journal of Biological Chemistry 280:12, pages 11648-11655.
Crossref
Crossref
Caterina Bendotti & Maria Teresa Carrì. (2004) Lessons from models of SOD1-linked familial ALS. Trends in Molecular Medicine 10:8, pages 393-400.
Crossref
Crossref
Lucie I. BruijnTimothy M. MillerDon W. Cleveland. (2004) UNRAVELING THE MECHANISMS INVOLVED IN MOTOR NEURON DEGENERATION IN ALS. Annual Review of Neuroscience 27:1, pages 723-749.
Crossref
Crossref
Kou Miyazaki, Tomoyuki Fujita, Toshinori Ozaki, Chiaki Kato, Yuka Kurose, Maya Sakamoto, Shinsuke Kato, Takeshi Goto, Yasuto Itoyama, Masashi Aoki & Akira Nakagawara. (2004) NEDL1, a Novel Ubiquitin-protein Isopeptide Ligase for Dishevelled-1, Targets Mutant Superoxide Dismutase-1. Journal of Biological Chemistry 279:12, pages 11327-11335.
Crossref
Crossref
P. Andreas Jonsson, Karin Ernhill, Peter M. Andersen, Daniel Bergemalm, Thomas Brännström, Ole Gredal, Peter Nilsson & Stefan L. Marklund. (2004) Minute quantities of misfolded mutant superoxide dismutase‐1 cause amyotrophic lateral sclerosis. Brain 127:1, pages 73-88.
Crossref
Crossref
Cui Zhen He & Arthur P. Hays. (2004) Expression of peripherin in ubiquinated inclusions of amyotrophic lateral sclerosis. Journal of the Neurological Sciences 217:1, pages 47-54.
Crossref
Crossref
P. B. Stathopulos, J. A. O. Rumfeldt, G. A. Scholz, R. A. Irani, H. E. Frey, R. A. Hallewell, J. R. Lepock & E. M. Meiering. (2003)
Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates
in vitro
. Proceedings of the National Academy of Sciences 100:12, pages 7021-7026.
Crossref
Crossref
Byron CaugheyPeter T. LansburyJr.Jr.. (2003)
P
ROTOFIBRILS
, P
ORES
, F
IBRILS, AND
N
EURODEGENERATION
: Separating the Responsible Protein Aggregates from The Innocent Bystanders
. Annual Review of Neuroscience 26:1, pages 267-298.
Crossref
Crossref
D Majoor-Krakauer, PJ Willems & A Hofman. (2003) Genetic epidemiology of amyotrophic lateral sclerosis. Clinical Genetics 63:2, pages 83-101.
Crossref
Crossref
Joan Selverstone Valentine. (2002) Do oxidatively modified proteins cause ALS?. Free Radical Biology and Medicine 33:10, pages 1314-1320.
Crossref
Crossref
P. L. McGeer & E. G. McGeer. (2002) Inflammatory processes in amyotrophic lateral sclerosis. Muscle & Nerve 26:4, pages 459-470.
Crossref
Crossref
Jun-ichi Niwa, Shinsuke Ishigaki, Nozomi Hishikawa, Masahiko Yamamoto, Manabu Doyu, Shigeo Murata, Keiji Tanaka, Naoyuki Taniguchi & Gen Sobue. (2002) Dorfin Ubiquitylates Mutant SOD1 and Prevents Mutant SOD1-mediated Neurotoxicity. Journal of Biological Chemistry 277:39, pages 36793-36798.
Crossref
Crossref
Asao Hirano. (2002) Changes in age‐associated neurodegenerative diseases in the last half century. Geriatrics & Gerontology International 2:3, pages 122-126.
Crossref
Crossref
A. Naini, O. Musumeci, L. Hayes, F. Pallotti, M. Del Bene & H. Mitsumoto. (2002) Identification of a novel mutation in Cu/Zn superoxide dismutase gene associated with familial amyotrophic lateral sclerosis. Journal of the Neurological Sciences 198:1-2, pages 17-19.
Crossref
Crossref
Shinsuke Kato, Kenji NakashimaSeikoh HoriuchiRyoji NagaiDon W. Cleveland, Jian Liu, Asao Hirano, Miki Takikawa, Masako KatoImaharu NakanoSaburo SakodaKohtaro AsayamaEisaku Ohama. (2008) Formation of advanced glycation end‐product‐modified superoxide dismutase‐1 (SOD1) is one of the mechanisms responsible for inclusions common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutation, and transgenic mice expressing human SOD1 gene mutation. Neuropathology 21:1, pages 67-81.
Crossref
Crossref