TY - JOUR AU - Andersen, P. M. AU - Al-Chalabi, A. PY - 2011 DA - 2011// TI - Clinical genetics of amyotrophic lateral sclerosis: what do we really know? JO - Nat Rev Neurol. VL - 7 UR - https://doi.org/10.1038/nrneurol.2011.150 DO - 10.1038/nrneurol.2011.150 ID - Andersen2011 ER - TY - JOUR AU - Rosen, D. R. AU - Siddique, T. AU - Patterson, D. AU - Figlewicz, D. A. AU - Sapp, P. AU - Hentati, A. PY - 1993 DA - 1993// TI - Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis JO - Nature. VL - 362 UR - https://doi.org/10.1038/362059a0 DO - 10.1038/362059a0 ID - Rosen1993 ER - TY - JOUR AU - Abel, O. AU - Powell, J. F. AU - Andersen, P. M. AU - Al-Chalabi, A. PY - 2012 DA - 2012// TI - ALSoD: A user-friendly online bioinformatics tool for amyotrophic lateral sclerosis genetics JO - Hum Mutat. VL - 33 UR - https://doi.org/10.1002/humu.22157 DO - 10.1002/humu.22157 ID - Abel2012 ER - TY - JOUR AU - Reaume, A. G. AU - Elliott, J. L. AU - Hoffman, E. K. AU - Kowall, N. W. AU - Ferrante, R. J. AU - Siwek, D. F. PY - 1996 DA - 1996// TI - Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury JO - Nat Genet. VL - 13 UR - https://doi.org/10.1038/ng0596-43 DO - 10.1038/ng0596-43 ID - Reaume1996 ER - TY - JOUR AU - Bruijn, L. I. AU - Houseweart, M. K. AU - Kato, S. AU - Anderson, K. L. AU - Anderson, S. D. AU - Ohama, E. PY - 1998 DA - 1998// TI - Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1 JO - Science. VL - 281 UR - https://doi.org/10.1126/science.281.5384.1851 DO - 10.1126/science.281.5384.1851 ID - Bruijn1998 ER - TY - JOUR AU - McCord, J. M. AU - Fridovich, I. PY - 1969 DA - 1969// TI - Superoxide dismutase. An enzymic function for erythrocuprein (hemocuprein) JO - J Biol Chem VL - 244 ID - McCord1969 ER - TY - JOUR AU - Furukawa, Y. AU - O'Halloran, T. V. PY - 2005 DA - 2005// TI - Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo, reduced form of SOD1, leading to unfolding and oxidative aggregation JO - J Biol Chem. VL - 280 UR - https://doi.org/10.1074/jbc.M500482200 DO - 10.1074/jbc.M500482200 ID - Furukawa2005 ER - TY - JOUR AU - Toichi, K. AU - Yamanaka, K. AU - Furukawa, Y. PY - 2013 DA - 2013// TI - Disulfide scrambling describes the oligomer formation of superoxide dismutase (SOD1) proteins in the familial form of amyotrophic lateral sclerosis JO - J Biol Chem. VL - 288 UR - https://doi.org/10.1074/jbc.M112.414235 DO - 10.1074/jbc.M112.414235 ID - Toichi2013 ER - TY - JOUR AU - Furukawa, Y. AU - Kaneko, K. AU - Yamanaka, K. AU - O'Halloran, T. V. AU - Nukina, N. PY - 2008 DA - 2008// TI - Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in familial form of ALS JO - J Biol Chem. VL - 283 UR - https://doi.org/10.1074/jbc.M802083200 DO - 10.1074/jbc.M802083200 ID - Furukawa2008 ER - TY - JOUR AU - Proctor, E. A. AU - Fee, L. AU - Tao, Y. AU - Redler, R. L. AU - Fay, J. M. AU - Zhang, Y. PY - 2016 DA - 2016// TI - Nonnative SOD1 trimer is toxic to motor neurons in a model of amyotrophic lateral sclerosis JO - Proc Natl Acad Sci USA VL - 113 UR - https://doi.org/10.1073/pnas.1516725113 DO - 10.1073/pnas.1516725113 ID - Proctor2016 ER - TY - JOUR AU - Banci, L. AU - Bertini, I. AU - Boca, M. AU - Calderone, V. AU - Cantini, F. AU - Girotto, S. PY - 2009 DA - 2009// TI - Structural and dynamic aspects related to oligomerization of apo SOD1 and its mutants JO - Proc Natl Acad Sci USA VL - 106 UR - https://doi.org/10.1073/pnas.0809845106 DO - 10.1073/pnas.0809845106 ID - Banci2009 ER - TY - JOUR AU - Luchinat, E. AU - Barbieri, L. AU - Rubino, J. T. AU - Kozyreva, T. AU - Cantini, F. AU - Banci, L. PY - 2014 DA - 2014// TI - In-cell NMR reveals potential precursor of toxic species from SOD1 fALS mutants JO - Nat Commun. VL - 5 UR - https://doi.org/10.1038/ncomms6502 DO - 10.1038/ncomms6502 ID - Luchinat2014 ER - TY - JOUR AU - Sekhar, A. AU - Rumfeldt, J. A. AU - Broom, H. R. AU - Doyle, C. M. AU - Bouvignies, G. AU - Meiering, E. M. PY - 2015 DA - 2015// TI - Thermal fluctuations of immature SOD1 lead to separate folding and misfolding pathways JO - eLife. VL - 4 UR - https://doi.org/10.7554/eLife.07296 DO - 10.7554/eLife.07296 ID - Sekhar2015 ER - TY - JOUR AU - Jonsson, P. A. AU - Graffmo, K. S. AU - Andersen, P. M. AU - Brannstrom, T. AU - Lindberg, M. AU - Oliveberg, M. PY - 2006 DA - 2006// TI - Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models JO - Brain. VL - 129 UR - https://doi.org/10.1093/brain/awh704 DO - 10.1093/brain/awh704 ID - Jonsson2006 ER - TY - JOUR AU - Wang, J. AU - Xu, G. AU - Gonzales, V. AU - Coonfield, M. AU - Fromholt, D. AU - Copeland, N. G. PY - 2002 DA - 2002// TI - Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site JO - Neurobiol Dis. VL - 10 UR - https://doi.org/10.1006/nbdi.2002.0498 DO - 10.1006/nbdi.2002.0498 ID - Wang2002 ER - TY - JOUR AU - Kerman, A. AU - Liu, H. N. AU - Croul, S. AU - Bilbao, J. AU - Rogaeva, E. AU - Zinman, L. PY - 2010 DA - 2010// TI - Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form JO - Acta Neuropathol. VL - 119 UR - https://doi.org/10.1007/s00401-010-0646-5 DO - 10.1007/s00401-010-0646-5 ID - Kerman2010 ER - TY - JOUR AU - Furukawa, Y. AU - Fu, R. AU - Deng, H. X. AU - Siddique, T. AU - O'Halloran, T. V. PY - 2006 DA - 2006// TI - Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice JO - Proc Natl Acad Sci USA VL - 103 UR - https://doi.org/10.1073/pnas.0602048103 DO - 10.1073/pnas.0602048103 ID - Furukawa2006 ER - TY - JOUR AU - Karch, C. M. AU - Borchelt, D. R. PY - 2008 DA - 2008// TI - A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis JO - J Biol Chem. VL - 283 UR - https://doi.org/10.1074/jbc.M800564200 DO - 10.1074/jbc.M800564200 ID - Karch2008 ER - TY - JOUR AU - Niwa, J. AU - Yamada, S. AU - Ishigaki, S. AU - Sone, J. AU - Takahashi, M. AU - Katsuno, M. PY - 2007 DA - 2007// TI - Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1 JO - J Biol Chem. VL - 282 UR - https://doi.org/10.1074/jbc.M704465200 DO - 10.1074/jbc.M704465200 ID - Niwa2007 ER - TY - JOUR AU - Yamanaka, K. AU - Chun, S. J. AU - Boillee, S. AU - Fujimori-Tonou, N. AU - Yamashita, H. AU - Gutmann, D. H. PY - 2008 DA - 2008// TI - Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis JO - Nat Neurosci. VL - 11 UR - https://doi.org/10.1038/nn2047 DO - 10.1038/nn2047 ID - Yamanaka2008 ER - TY - JOUR AU - Williamson, T. L. AU - Cleveland, D. W. PY - 1999 DA - 1999// TI - Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons JO - Nat Neurosci. VL - 2 UR - https://doi.org/10.1038/4553 DO - 10.1038/4553 ID - Williamson1999 ER - TY - JOUR AU - Ichikawa, T. AU - Ajiki, K. AU - Matsuura, J. AU - Misawa, H. PY - 1997 DA - 1997// TI - Localization of two cholinergic markers, choline acetyltransferase and vesicular acetylcholine transporter in the central nervous system of the rat: in situ hybridization histochemistry and immunohistochemistry JO - J Chem Neuroanat. VL - 13 UR - https://doi.org/10.1016/S0891-0618(97)00021-5 DO - 10.1016/S0891-0618(97)00021-5 ID - Ichikawa1997 ER - TY - JOUR AU - Deng, H. X. AU - Shi, Y. AU - Furukawa, Y. AU - Zhai, H. AU - Fu, R. AU - Liu, E. PY - 2006 DA - 2006// TI - Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria JO - Proc Natl Acad Sci USA VL - 103 UR - https://doi.org/10.1073/pnas.0602046103 DO - 10.1073/pnas.0602046103 ID - Deng2006 ER - TY - JOUR AU - Morisaki, Y. AU - Niikura, M. AU - Watanabe, M. AU - Onishi, K. AU - Tanabe, S. AU - Moriwaki, Y. PY - 2016 DA - 2016// TI - Selective Expression of Osteopontin in ALS-resistant Motor Neurons is a Critical Determinant of Late Phase Neurodegeneration Mediated by Matrix Metalloproteinase-9 JO - Sci Rep. VL - 6 UR - https://doi.org/10.1038/srep27354 DO - 10.1038/srep27354 ID - Morisaki2016 ER - TY - JOUR AU - Watanabe, M. AU - Dykes-Hoberg, M. AU - Culotta, V. C. AU - Price, D. L. AU - Wong, P. C. AU - Rothstein, J. D. PY - 2001 DA - 2001// TI - Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues JO - Neurobiol Dis. VL - 8 UR - https://doi.org/10.1006/nbdi.2001.0443 DO - 10.1006/nbdi.2001.0443 ID - Watanabe2001 ER - TY - JOUR AU - Nakamura, A. AU - Hineno, A. AU - Yoshida, K. AU - Sekijima, Y. AU - Hanaoka-Tachibana, N. AU - Takei, Y. PY - 2012 DA - 2012// TI - Marked intrafamilial phenotypic variation in a family with SOD1 C111Y mutation JO - Amyotroph Lateral Scler. VL - 13 UR - https://doi.org/10.3109/17482968.2012.656311 DO - 10.3109/17482968.2012.656311 ID - Nakamura2012 ER - TY - JOUR AU - Takei, Y. AU - Oguchi, K. AU - Koshihara, H. AU - Hineno, A. AU - Nakamura, A. AU - Ohara, S. PY - 2013 DA - 2013// TI - alpha-Synuclein coaggregation in familial amyotrophic lateral sclerosis with SOD1 gene mutation JO - Hum Pathol VL - 44 UR - https://doi.org/10.1016/j.humpath.2012.10.024 DO - 10.1016/j.humpath.2012.10.024 ID - Takei2013 ER - TY - JOUR AU - Rakhit, R. AU - Robertson, J. AU - Vande Velde, C. AU - Horne, P. AU - Ruth, D. M. AU - Griffin, J. PY - 2007 DA - 2007// TI - An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS JO - Nat Med. VL - 13 UR - https://doi.org/10.1038/nm1559 DO - 10.1038/nm1559 ID - Rakhit2007 ER - TY - JOUR AU - Swinnen, B. AU - Robberecht, W. PY - 2014 DA - 2014// TI - The phenotypic variability of amyotrophic lateral sclerosis JO - Nat Rev Neurol. VL - 10 UR - https://doi.org/10.1038/nrneurol.2014.184 DO - 10.1038/nrneurol.2014.184 ID - Swinnen2014 ER - TY - JOUR AU - Garbuzova-Davis, S. AU - Saporta, S. AU - Haller, E. AU - Kolomey, I. AU - Bennett, S. P. AU - Potter, H. PY - 2007 DA - 2007// TI - Evidence of compromised blood-spinal cord barrier in early and late symptomatic SOD1 mice modeling ALS JO - PLoS One. VL - 2 UR - https://doi.org/10.1371/journal.pone.0001205 DO - 10.1371/journal.pone.0001205 ID - Garbuzova-Davis2007 ER - TY - JOUR AU - Zang, D. W. AU - Cheema, S. S. PY - 2002 DA - 2002// TI - Degeneration of corticospinal and bulbospinal systems in the superoxide dismutase 1(G93A G1H) transgenic mouse model of familial amyotrophic lateral sclerosis JO - Neurosci Lett. VL - 332 UR - https://doi.org/10.1016/S0304-3940(02)00944-8 DO - 10.1016/S0304-3940(02)00944-8 ID - Zang2002 ER - TY - JOUR AU - Dal Canto, M. C. AU - Gurney, M. E. PY - 1995 DA - 1995// TI - Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu, Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS) JO - Brain Res. VL - 676 UR - https://doi.org/10.1016/0006-8993(95)00063-V DO - 10.1016/0006-8993(95)00063-V ID - Dal Canto1995 ER - TY - JOUR AU - Metcalf, C. W. AU - Hirano, A. PY - 1971 DA - 1971// TI - Amyotrophic lateral sclerosis. Clinicopathological studies of a family JO - Arch Neurol VL - 24 UR - https://doi.org/10.1001/archneur.1971.00480360052006 DO - 10.1001/archneur.1971.00480360052006 ID - Metcalf1971 ER - TY - JOUR AU - Boillee, S. AU - Yamanaka, K. AU - Lobsiger, C. S. AU - Copeland, N. G. AU - Jenkins, N. A. AU - Kassiotis, G. PY - 2006 DA - 2006// TI - Onset and progression in inherited ALS determined by motor neurons and microglia JO - Science. VL - 312 UR - https://doi.org/10.1126/science.1123511 DO - 10.1126/science.1123511 ID - Boillee2006 ER - TY - JOUR AU - Kang, S. H. AU - Li, Y. AU - Fukaya, M. AU - Lorenzini, I. AU - Cleveland, D. W. AU - Ostrow, L. W. PY - 2013 DA - 2013// TI - Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis JO - Nat Neurosci. VL - 16 UR - https://doi.org/10.1038/nn.3357 DO - 10.1038/nn.3357 ID - Kang2013 ER - TY - JOUR AU - Rodriguez, J. A. AU - Shaw, B. F. AU - Durazo, A. AU - Sohn, S. H. AU - Doucette, P. A. AU - Nersissian, A. M. PY - 2005 DA - 2005// TI - Destabilization of apoprotein is insufficient to explain Cu, Zn-superoxide dismutase-linked ALS pathogenesis JO - Proc Natl Acad Sci USA VL - 102 UR - https://doi.org/10.1073/pnas.0502515102 DO - 10.1073/pnas.0502515102 ID - Rodriguez2005 ER - TY - JOUR AU - Banci, L. AU - Bertini, I. AU - Durazo, A. AU - Girotto, S. AU - Gralla, E. B. AU - Martinelli, M. PY - 2007 DA - 2007// TI - Metal-free superoxide dismutase forms soluble oligomers under physiological conditions: a possible general mechanism for familial ALS JO - Proc Natl Acad Sci USA VL - 104 UR - https://doi.org/10.1073/pnas.0704307104 DO - 10.1073/pnas.0704307104 ID - Banci2007 ER - TY - JOUR AU - Rotunno, M. S. AU - Bosco, D. A. PY - 2013 DA - 2013// TI - An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis JO - Front Cell Neurosci. VL - 7 UR - https://doi.org/10.3389/fncel.2013.00253 DO - 10.3389/fncel.2013.00253 ID - Rotunno2013 ER - TY - JOUR AU - Sabado, J. AU - Casanovas, A. AU - Hernandez, S. AU - Piedrafita, L. AU - Hereu, M. AU - Esquerda, J. E. PY - 2013 DA - 2013// TI - Immunodetection of disease-associated conformers of mutant cu/zn superoxide dismutase 1 selectively expressed in degenerating neurons in amyotrophic lateral sclerosis JO - J Neuropathol Exp Neurol. VL - 72 UR - https://doi.org/10.1097/NEN.0b013e318297fd10 DO - 10.1097/NEN.0b013e318297fd10 ID - Sabado2013 ER - TY - JOUR AU - Gros-Louis, F. AU - Soucy, G. AU - Lariviere, R. AU - Julien, J. P. PY - 2010 DA - 2010// TI - Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS JO - J Neurochem. VL - 113 ID - Gros-Louis2010 ER - TY - JOUR AU - Rotunno, M. S. AU - Auclair, J. R. AU - Maniatis, S. AU - Shaffer, S. A. AU - Agar, J. AU - Bosco, D. A. PY - 2014 DA - 2014// TI - Identification of a misfolded region in superoxide dismutase 1 that is exposed in amyotrophic lateral sclerosis JO - J Biol Chem. VL - 289 UR - https://doi.org/10.1074/jbc.M114.581801 DO - 10.1074/jbc.M114.581801 ID - Rotunno2014 ER - TY - JOUR AU - Urushitani, M. AU - Ezzi, S. A. AU - Julien, J. P. PY - 2007 DA - 2007// TI - Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis JO - Proc Natl Acad Sci USA VL - 104 UR - https://doi.org/10.1073/pnas.0606201104 DO - 10.1073/pnas.0606201104 ID - Urushitani2007 ER - TY - JOUR AU - Brotherton, T. E. AU - Li, Y. AU - Cooper, D. AU - Gearing, M. AU - Julien, J. P. AU - Rothstein, J. D. PY - 2012 DA - 2012// TI - Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS JO - Proc Natl Acad Sci USA VL - 109 UR - https://doi.org/10.1073/pnas.1115009109 DO - 10.1073/pnas.1115009109 ID - Brotherton2012 ER - TY - JOUR AU - Okamoto, Y. AU - Ihara, M. AU - Urushitani, M. AU - Yamashita, H. AU - Kondo, T. AU - Tanigaki, A. PY - 2011 DA - 2011// TI - An autopsy case of SOD1-related ALS with TDP-43 positive inclusions JO - Neurology. VL - 77 UR - https://doi.org/10.1212/WNL.0b013e31823a0cfc DO - 10.1212/WNL.0b013e31823a0cfc ID - Okamoto2011 ER - TY - JOUR AU - Khare, S. D. AU - Caplow, M. AU - Dokholyan, N. V. PY - 2004 DA - 2004// TI - The rate and equilibrium constants for a multistep reaction sequence for the aggregation of superoxide dismutase in amyotrophic lateral sclerosis JO - Proc Natl Acad Sci USA VL - 101 UR - https://doi.org/10.1073/pnas.0406650101 DO - 10.1073/pnas.0406650101 ID - Khare2004 ER - TY - JOUR AU - Jonsson, P. A. AU - Ernhill, K. AU - Andersen, P. M. AU - Bergemalm, D. AU - Brannstrom, T. AU - Gredal, O. PY - 2004 DA - 2004// TI - Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis JO - Brain. VL - 127 UR - https://doi.org/10.1093/brain/awh005 DO - 10.1093/brain/awh005 ID - Jonsson2004 ER - TY - JOUR AU - Forsberg, K. AU - Andersen, P. M. AU - Marklund, S. L. AU - Brannstrom, T. PY - 2011 DA - 2011// TI - Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis JO - Acta Neuropathol. VL - 121 UR - https://doi.org/10.1007/s00401-011-0805-3 DO - 10.1007/s00401-011-0805-3 ID - Forsberg2011 ER - TY - JOUR AU - Forsberg, K. AU - Jonsson, P. A. AU - Andersen, P. M. AU - Bergemalm, D. AU - Graffmo, K. S. AU - Hultdin, M. PY - 2010 DA - 2010// TI - Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients JO - PLoS One. VL - 5 UR - https://doi.org/10.1371/journal.pone.0011552 DO - 10.1371/journal.pone.0011552 ID - Forsberg2010 ER - TY - JOUR AU - Liu, H. N. AU - Sanelli, T. AU - Horne, P. AU - Pioro, E. P. AU - Strong, M. J. AU - Rogaeva, E. PY - 2009 DA - 2009// TI - Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis JO - Ann Neurol. VL - 66 UR - https://doi.org/10.1002/ana.21704 DO - 10.1002/ana.21704 ID - Liu2009 ER - TY - JOUR AU - Stewart, H. G. AU - Mackenzie, I. R. AU - Eisen, A. AU - Brannstrom, T. AU - Marklund, S. L. AU - Andersen, P. M. PY - 2006 DA - 2006// TI - Clinicopathological phenotype of ALS with a novel G72C SOD1 gene mutation mimicking a myopathy JO - Muscle Nerve. VL - 33 UR - https://doi.org/10.1002/mus.20495 DO - 10.1002/mus.20495 ID - Stewart2006 ER - TY - JOUR AU - Chattopadhyay, M. AU - Durazo, A. AU - Sohn, S. H. AU - Strong, C. D. AU - Gralla, E. B. AU - Whitelegge, J. P. PY - 2008 DA - 2008// TI - Initiation and elongation in fibrillation of ALS-linked superoxide dismutase JO - Proc Natl Acad Sci USA VL - 105 UR - https://doi.org/10.1073/pnas.0807058105 DO - 10.1073/pnas.0807058105 ID - Chattopadhyay2008 ER - TY - JOUR AU - Oztug Durer, Z. A. AU - Cohlberg, J. A. AU - Dinh, P. AU - Padua, S. AU - Ehrenclou, K. AU - Downes, S. PY - 2009 DA - 2009// TI - Loss of metal ions, disulfide reduction and mutations related to familial ALS promote formation of amyloid-like aggregates from superoxide dismutase JO - PLoS One. VL - 4 UR - https://doi.org/10.1371/journal.pone.0005004 DO - 10.1371/journal.pone.0005004 ID - Oztug Durer2009 ER - TY - JOUR AU - Rakhit, R. AU - Cunningham, P. AU - Furtos-Matei, A. AU - Dahan, S. AU - Qi, X. F. AU - Crow, J. P. PY - 2002 DA - 2002// TI - Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis JO - J Biol Chem. VL - 277 UR - https://doi.org/10.1074/jbc.M207356200 DO - 10.1074/jbc.M207356200 ID - Rakhit2002 ER - TY - JOUR AU - Stathopulos, P. B. AU - Rumfeldt, J. A. AU - Scholz, G. A. AU - Irani, R. A. AU - Frey, H. E. AU - Hallewell, R. A. PY - 2003 DA - 2003// TI - Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro JO - Proc Natl Acad Sci USA VL - 100 UR - https://doi.org/10.1073/pnas.1237797100 DO - 10.1073/pnas.1237797100 ID - Stathopulos2003 ER - TY - JOUR AU - Furukawa, Y. AU - Anzai, I. AU - Akiyama, S. AU - Imai, M. AU - Cruz, F. J. AU - Saio, T. PY - 2016 DA - 2016// TI - Conformational Disorder of the Most Immature Cu, Zn-Superoxide Dismutase Leading to Amyotrophic Lateral Sclerosis JO - J Biol Chem. VL - 291 UR - https://doi.org/10.1074/jbc.M115.683763 DO - 10.1074/jbc.M115.683763 ID - Furukawa2016 ER - TY - JOUR AU - Furukawa, Y. AU - Kaneko, K. AU - Yamanaka, K. AU - Nukina, N. PY - 2010 DA - 2010// TI - Mutation-dependent polymorphism of Cu, Zn-superoxide dismutase aggregates in the familial form of amyotrophic lateral sclerosis JO - J Biol Chem. VL - 285 UR - https://doi.org/10.1074/jbc.M110.113597 DO - 10.1074/jbc.M110.113597 ID - Furukawa2010 ER - TY - JOUR AU - Niwa, J. AU - Ishigaki, S. AU - Hishikawa, N. AU - Yamamoto, M. AU - Doyu, M. AU - Murata, S. PY - 2002 DA - 2002// TI - Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity JO - J Biol Chem. VL - 277 UR - https://doi.org/10.1074/jbc.M206559200 DO - 10.1074/jbc.M206559200 ID - Niwa2002 ER - TY - JOUR AU - Ferri, A. AU - Cozzolino, M. AU - Crosio, C. AU - Nencini, M. AU - Casciati, A. AU - Gralla, E. B. PY - 2006 DA - 2006// TI - Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials JO - Proc Natl Acad Sci USA VL - 103 UR - https://doi.org/10.1073/pnas.0605814103 DO - 10.1073/pnas.0605814103 ID - Ferri2006 ER - TY - JOUR AU - Jaarsma, D. AU - Haasdijk, E. D. AU - Grashorn, J. A. AU - Hawkins, R. AU - Duijn, W. AU - Verspaget, H. W. PY - 2000 DA - 2000// TI - Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1 JO - Neurobiol Dis. VL - 7 UR - https://doi.org/10.1006/nbdi.2000.0299 DO - 10.1006/nbdi.2000.0299 ID - Jaarsma2000 ER - TY - JOUR AU - Furukawa, Y. PY - 2012 DA - 2012// TI - Pathological roles of wild-type cu, zn-superoxide dismutase in amyotrophic lateral sclerosis JO - Neurol Res Int. VL - 2012 UR - https://doi.org/10.1155/2012/323261 DO - 10.1155/2012/323261 ID - Furukawa2012 ER -