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Perspective

SOD1‐associated ALS: A promising system for elucidating the origin of protein‐misfolding disease

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Pages 354-364 | Received 01 Aug 2008, Published online: 07 Sep 2010

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Muthusamy Sureshan, Dhamodharan Prabhu & Saraboji Kadhirvel. (2023) Computational identification and experimental validation of anti-filarial lead molecules targeting metal binding/substrate channel residues of Cu/Zn SOD1 from Wuchereria bancrofti. Journal of Biomolecular Structure and Dynamics 41:18, pages 8715-8728.
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Dharma Rao Tompa & Saraboji Kadhirvel. (2018) Molecular dynamics of a far positioned SOD1 mutant V14M reveals pathogenic misfolding behavior. Journal of Biomolecular Structure and Dynamics 36:15, pages 4085-4098.
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Amresh Prakash, Vijay Kumar, Preeti Pandey, Deepak R. Bharti, Poonam Vishwakarma, Ruhar Singh, Md. Imtaiyaz Hassan & Andrew M. Lynn. (2018) Solvent sensitivity of protein aggregation in Cu, Zn superoxide dismutase: a molecular dynamics simulation study. Journal of Biomolecular Structure and Dynamics 36:10, pages 2605-2617.
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Claudia Ricci, Michele Benigni, Stefania Battistini, Giuseppe Greco, Antonio Torzini & Fabio Giannini. (2010) A novel exon 1 mutation (G10R) in the SOD1 gene in a patient with familial ALS. Amyotrophic Lateral Sclerosis 11:5, pages 481-485.
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Elaheh Mavadat, Bagher Seyedalipour, Saman Hosseinkhani & Abasalt Hosseinzadeh Colagar. (2023) Role of charged residues of the “electrostatic loop” of hSOD1 in promotion of aggregation: Implications for the mechanism of ALS-associated mutations under amyloidogenic conditions. International Journal of Biological Macromolecules 244, pages 125289.
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Hamza Dakhil Zaji, Bagher Seyedalipour, Haider Munzer Hanun, Payam Baziyar, Saman Hosseinkhani & Mona Akhlaghi. (2023) Computational insight into in silico analysis and molecular dynamics simulation of the dimer interface residues of ALS-linked hSOD1 forms in apo/holo states: a combined experimental and bioinformatic perspective. 3 Biotech 13:3.
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Mikayla L. Brown, Luke McAlary, Jeremy S. Lum, Natalie E. Farrawell & Justin J. Yerbury. (2022) Cells Overexpressing ALS-Associated SOD1 Variants Are Differentially Susceptible to CuATSM-Associated Toxicity. ACS Chemical Neuroscience 13:16, pages 2371-2379.
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Pauline Elisabeth Kolkwitz, Jeannine Mohrlüder & Dieter Willbold. (2022) Inhibition of Polyglutamine Misfolding with D-Enantiomeric Peptides Identified by Mirror Image Phage Display Selection. Biomolecules 12:2, pages 157.
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Karen E. Butler, Yoshihiko Takinami, Adam Rainczuk, Erin S. Baker & Blaine R. Roberts. (2021) Utilizing Ion Mobility-Mass Spectrometry to Investigate the Unfolding Pathway of Cu/Zn Superoxide Dismutase. Frontiers in Chemistry 9.
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Dharma Rao Tompa, Sureshan Muthusamy, Srimari Srikanth & Saraboji Kadhirvel. (2020) Molecular dynamics of far positioned surface mutations of Cu/Zn SOD1 promotes altered structural stability and metal-binding site: Structural clues to the pathogenesis of amyotrophic lateral sclerosis. Journal of Molecular Graphics and Modelling 100, pages 107678.
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Jelena Cveticanin, Tridib Mondal, Elizabeth M. Meiering, Michal Sharon & Amnon Horovitz. (2020) Insight into the Autosomal-Dominant Inheritance Pattern of SOD1-Associated ALS from Native Mass Spectrometry. Journal of Molecular Biology 432:23, pages 5995-6002.
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Lucas S. Dantas, Lucas G. Viviani, Alex Inague, Erika Piccirillo, Leandro de Rezende, Graziella E. Ronsein, Ohara Augusto, Marisa H.G. Medeiros, Antonia T. do Amaral & Sayuri Miyamoto. (2020) Lipid aldehyde hydrophobicity affects apo-SOD1 modification and aggregation. Free Radical Biology and Medicine 156, pages 157-167.
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Jaime Santos, Jordi Pujols, Irantzu Pallarès, Valentín Iglesias & Salvador Ventura. (2020) Computational prediction of protein aggregation: Advances in proteomics, conformation-specific algorithms and biotechnological applications. Computational and Structural Biotechnology Journal 18, pages 1403-1413.
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Jaime Santos, Valentín Iglesias & Salvador Ventura. 2020. Oligomerization in Health and Disease: From Enzymes to G Protein-Coupled Receptors. Oligomerization in Health and Disease: From Enzymes to G Protein-Coupled Receptors 43 83 .
Cameron Wells, Samuel E. Brennan, Matt Keon & Nitin K. Saksena. (2019) Prionoid Proteins in the Pathogenesis of Neurodegenerative Diseases. Frontiers in Molecular Neuroscience 12.
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David N. Bunck, Beatriz Atsavapranee, Anna K. Museth, David VanderVelde & James R. Heath. (2018) Modulating the Folding Landscape of Superoxide Dismutase 1 with Targeted Molecular Binders. Angewandte Chemie International Edition 57:21, pages 6212-6215.
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David N. Bunck, Beatriz Atsavapranee, Anna K. Museth, David VanderVelde & James R. Heath. (2018) Modulating the Folding Landscape of Superoxide Dismutase 1 with Targeted Molecular Binders. Angewandte Chemie 130:21, pages 6320-6323.
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Caroline Sibilla & Anne Bertolotti. (2017) Prion Properties of SOD1 in Amyotrophic Lateral Sclerosis and Potential Therapy. Cold Spring Harbor Perspectives in Biology 9:10, pages a024141.
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Jens Danielsson & Mikael Oliveberg. (2017) Comparing protein behaviour in vitro and in vivo , what does the data really tell us?. Current Opinion in Structural Biology 42, pages 129-135.
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Luke McAlary, J. Andrew Aquilina & Justin J. Yerbury. (2016) Susceptibility of Mutant SOD1 to Form a Destabilized Monomer Predicts Cellular Aggregation and Toxicity but Not In vitro Aggregation Propensity. Frontiers in Neuroscience 10.
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Xin Gen LeiJian-Hong ZhuWen-Hsing ChengYongping BaoYe-Shih HoAmit R. ReddiArne HolmgrenElias S. J. Arnér. (2016) Paradoxical Roles of Antioxidant Enzymes: Basic Mechanisms and Health Implications. Physiological Reviews 96:1, pages 307-364.
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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.
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Emmanuel Sapin, Kenneth A De Jong & Amarda Shehu. (2015) Evolutionary search strategies for efficient sample-based representations of multiple-basin protein energy landscapes. Evolutionary search strategies for efficient sample-based representations of multiple-basin protein energy landscapes.
Indrajit DasAgnieszka KrzyzosiakKim SchneiderLawrence WrabetzMaurizio D’AntonioNicholas BarryAnna SigurdardottirAnne Bertolotti. (2015) Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit. Science 348:6231, pages 239-242.
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Sílvia G. Estácio, Sónia S. Leal, Joana S. Cristóvão, Patrícia F.N. Faísca & Cláudio M. Gomes. (2015) Calcium binding to gatekeeper residues flanking aggregation-prone segments underlies non-fibrillar amyloid traits in superoxide dismutase 1 (SOD1). Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics 1854:2, pages 118-126.
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Angel L. Pey, Clare F. Megarity & David J. Timson. (2014) FAD binding overcomes defects in activity and stability displayed by cancer-associated variants of human NQO1. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1842:11, pages 2163-2173.
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Angel L. Pey. (2013) Protein homeostasis disorders of key enzymes of amino acids metabolism: mutation-induced protein kinetic destabilization and new therapeutic strategies. Amino Acids 45:6, pages 1331-1341.
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T. Schmidlin, K. Ploeger, A. L. Jonsson & V. Daggett. (2013) Early steps in thermal unfolding of superoxide dismutase 1 are similar to the conformational changes associated with the ALS-associated A4V mutation. Protein Engineering Design and Selection 26:8, pages 503-513.
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Angel L. Pey, Noel Mesa-Torres, Laurent R. Chiarelli & Giovanna Valentini. (2013) Structural and Energetic Basis of Protein Kinetic Destabilization in Human Phosphoglycerate Kinase 1 Deficiency. Biochemistry 52:7, pages 1160-1170.
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J. Nicole Burns, Katherine C. Turnage, Chandler A. Walker & Raquel L. Lieberman. (2011) The Stability of Myocilin Olfactomedin Domain Variants Provides New Insight into Glaucoma as a Protein Misfolding Disorder. Biochemistry 50:26, pages 5824-5833.
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Lucia Banci, Ivano Bertini, Olga Blaževitš, Francesca Cantini, Moreno Lelli, Claudio Luchinat, Jiafei Mao & Miguela Vieru. (2010) NMR Characterization of a “Fibril-Ready” State of Demetalated Wild-Type Superoxide Dismutase. Journal of the American Chemical Society 133:2, pages 345-349.
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André Bento-Abreu, Philip Van Damme, Ludo Van Den Bosch & Wim Robberecht. (2010) The neurobiology of amyotrophic lateral sclerosis. European Journal of Neuroscience 31:12, pages 2247-2265.
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Christian Münch & Anne Bertolotti. (2010) Exposure of Hydrophobic Surfaces Initiates Aggregation of Diverse ALS-Causing Superoxide Dismutase-1 Mutants. Journal of Molecular Biology 399:3, pages 512-525.
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J.J.P. Perry, D.S. Shin, E.D. Getzoff & J.A. Tainer. (2010) The structural biochemistry of the superoxide dismutases. Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics 1804:2, pages 245-262.
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