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PDBsum entry 1spd

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protein metals Protein-protein interface(s) links
Oxidoreductase PDB id
1spd
Jmol
Contents
Protein chain
154 a.a. *
Metals
_ZN ×2
_CU ×2
* Residue conservation analysis
PDB id:
1spd
Name: Oxidoreductase
Title: Amyotrophic lateral sclerosis and structural defects in cu,z superoxide dismutase
Structure: Superoxide dismutase. Chain: a, b. Engineered: yes
Source: Homo sapiens. Human. Organism_taxid: 9606
Biol. unit: Hexamer (from PQS)
Resolution:
2.40Å     R-factor:   0.224    
Authors: H.E.Parge,J.A.Tainer
Key ref: H.X.Deng et al. (1993). Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase. Science, 261, 1047-1051. PubMed id: 8351519 DOI: 10.1126/science.8351519
Date:
21-Jul-93     Release date:   30-Apr-94    
PROCHECK
Go to PROCHECK summary
 Headers
 References

Protein chains
Pfam   ArchSchema ?
P00441  (SODC_HUMAN) -  Superoxide dismutase [Cu-Zn]
Seq:
Struc:
154 a.a.
153 a.a.
Key:    PfamA domain  Secondary structure  CATH domain

 Enzyme reactions 
   Enzyme class: E.C.1.15.1.1  - Superoxide dismutase.
[IntEnz]   [ExPASy]   [KEGG]   [BRENDA]
      Reaction: 2 superoxide + 2 H+ = O2 + H2O2
2 × superoxide
+ 2 × H(+)
= O(2)
+ H(2)O(2)
      Cofactor: Fe cation or Mn(2+) or (Zn(2+) and Cu cation)
Molecule diagrams generated from .mol files obtained from the KEGG ftp site
 Gene Ontology (GO) functional annotation 
  GO annot!
  Cellular component     extracellular region   20 terms 
  Biological process     cellular response to potassium ion   66 terms 
  Biochemical function     antioxidant activity     13 terms  

 

 
    Added reference    
 
 
DOI no: 10.1126/science.8351519 Science 261:1047-1051 (1993)
PubMed id: 8351519  
 
 
Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase.
H.X.Deng, A.Hentati, J.A.Tainer, Z.Iqbal, A.Cayabyab, W.Y.Hung, E.D.Getzoff, P.Hu, B.Herzfeldt, R.P.Roos.
 
  ABSTRACT  
 
Single-site mutants in the Cu,Zn superoxide dismutase (SOD) gene (SOD1) occur in patients with the fatal neurodegenerative disorder familial amyotrophic lateral sclerosis (FALS). Complete screening of the SOD1 coding region revealed that the mutation Ala4 to Val in exon 1 was the most frequent one; mutations were identified in exons 2, 4, and 5 but not in the active site region formed by exon 3. The 2.4 A crystal structure of human SOD, along with two other SOD structures, established that all 12 observed FALS mutant sites alter conserved interactions critical to the beta-barrel fold and dimer contact, rather than catalysis. Red cells from heterozygotes had less than 50 percent normal SOD activity, consistent with a structurally defective SOD dimer. Thus, defective SOD is linked to motor neuron death and carries implications for understanding and possible treatment of FALS.
 

Literature references that cite this PDB file's key reference

  PubMed id Reference
21331408 C.M.Yang (2011).
Biometal binding-site mimicry with modular, hetero-bifunctionally modified architecture encompassing a Trp/His motif: insights into spatiotemporal noncovalent interactions from a comparative spectroscopic study.
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21108037 F.J.Miana-Mena, C.González-Mingot, P.Larrodé, M.J.Muñoz, S.Oliván, L.Fuentes-Broto, E.Martínez-Ballarín, R.J.Reiter, R.Osta, and J.J.García (2011).
Monitoring systemic oxidative stress in an animal model of amyotrophic lateral sclerosis.
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21270817 G.Liu, N.Hosomi, H.Hitomi, N.Pelisch, H.Fu, H.Masugata, K.Murao, M.Ueno, M.Matsumoto, and A.Nishiyama (2011).
Angiotensin II induces human astrocyte senescence through reactive oxygen species production.
  Hypertens Res, 34, 479-483.  
21329474 G.Zhao, X.Yin, D.Wu, S.Mao, H.Yin, and B.Zhang (2011).
Clinical features and cu/zn superoxide dismutase gene mutations in two mainland chinese families with amyotrophic lateral sclerosis.
  Int J Neurosci, 121, 191-195.  
21354101 H.K.Kim, Y.W.Chung, P.B.Chock, and M.B.Yim (2011).
Effect of CCS on the accumulation of FALS SOD1 mutant-containing aggregates and on mitochondrial translocation of SOD1 mutants: implication of a free radical hypothesis.
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21857683 H.X.Deng, W.Chen, S.T.Hong, K.M.Boycott, G.H.Gorrie, N.Siddique, Y.Yang, F.Fecto, Y.Shi, H.Zhai, H.Jiang, M.Hirano, E.Rampersaud, G.H.Jansen, S.Donkervoort, E.H.Bigio, B.R.Brooks, K.Ajroud, R.L.Sufit, J.L.Haines, E.Mugnaini, M.A.Pericak-Vance, and T.Siddique (2011).
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia.
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21414382 K.Jomova, and M.Valko (2011).
Advances in metal-induced oxidative stress and human disease.
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20924670 S.S.Yi, I.K.Hwang, D.W.Kim, J.H.Shin, S.M.Nam, J.H.Choi, C.H.Lee, M.H.Won, J.K.Seong, and Y.S.Yoon (2011).
The chronological characteristics of SOD1 activity and inflammatory response in the hippocampi of STZ-induced type 1 diabetic rats.
  Neurochem Res, 36, 117-128.  
21241159 T.D.Heiman-Patterson, R.B.Sher, E.A.Blankenhorn, G.Alexander, J.S.Deitch, C.B.Kunst, N.Maragakis, and G.Cox (2011).
Effect of genetic background on phenotype variability in transgenic mouse models of amyotrophic lateral sclerosis: A window of opportunity in the search for genetic modifiers.
  Amyotroph Lateral Scler, 12, 79-86.  
21241794 Z.Jiang, Z.Hu, L.Zeng, W.Lu, H.Zhang, T.Li, and H.Xiao (2011).
The role of the Golgi apparatus in oxidative stress: is this organelle less significant than mitochondria?
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20076970 A.H.Li, B.K.Na, S.K.Ahn, S.H.Cho, J.H.Pak, Y.K.Park, and T.S.Kim (2010).
Functional expression and characterization of a cytosolic copper/zinc-superoxide dismutase of Spirometra erinacei.
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20382740 A.M.El-Kadi, V.Bros-Facer, W.Deng, A.Philpott, E.Stoddart, G.Banks, G.S.Jackson, E.M.Fisher, M.R.Duchen, L.Greensmith, A.L.Moore, and M.Hafezparast (2010).
The legs at odd angles (Loa) mutation in cytoplasmic dynein ameliorates mitochondrial function in SOD1G93A mouse model for motor neuron disease.
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20874803 C.F.Meehan, M.Moldovan, S.L.Marklund, K.S.Graffmo, J.B.Nielsen, and H.Hultborn (2010).
Intrinsic properties of lumbar motor neurones in the adult G127insTGGG superoxide dismutase-1 mutant mouse in vivo: evidence for increased persistent inward currents.
  Acta Physiol (Oxf), 200, 361-376.  
20399791 C.Münch, and A.Bertolotti (2010).
Exposure of hydrophobic surfaces initiates aggregation of diverse ALS-causing superoxide dismutase-1 mutants.
  J Mol Biol, 399, 512-525.  
20669189 C.M.Yang, and J.Zhang (2010).
Insights into intramolecular Trp and His side-chain orientation and stereospecific π interactions surrounding metal centers: an investigation using protein metal-site mimicry in solution.
  Chemistry, 16, 10854-10865.  
20331403 C.Ricci, M.Benigni, S.Battistini, G.Greco, A.Torzini, and F.Giannini (2010).
A novel exon 1 mutation (G10R) in the SOD1 gene in a patient with familial ALS.
  Amyotroph Lateral Scler, 11, 481-485.  
20213043 H.T.Li, M.Jiao, J.Chen, and Y.Liang (2010).
Roles of zinc and copper in modulating the oxidative refolding of bovine copper, zinc superoxide dismutase.
  Acta Biochim Biophys Sin (Shanghai), 42, 183-194.  
20361056 H.Zhou, C.Huang, H.Chen, D.Wang, C.P.Landel, P.Y.Xia, R.Bowser, Y.J.Liu, and X.G.Xia (2010).
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
  PLoS Genet, 6, e1000887.  
20221404 J.C.Stevens, R.Chia, W.T.Hendriks, V.Bros-Facer, J.van Minnen, J.E.Martin, G.S.Jackson, L.Greensmith, G.Schiavo, and E.M.Fisher (2010).
Modification of superoxide dismutase 1 (SOD1) properties by a GFP tag--implications for research into amyotrophic lateral sclerosis (ALS).
  PLoS One, 5, e9541.  
20162303 J.J.Sager, Q.Bai, and E.A.Burton (2010).
Transgenic zebrafish models of neurodegenerative diseases.
  Brain Struct Funct, 214, 285-302.  
21098299 J.R.Auclair, K.J.Boggio, G.A.Petsko, D.Ringe, and J.N.Agar (2010).
Strategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosis.
  Proc Natl Acad Sci U S A, 107, 21394-21399.  
20808835 J.Yin, S.Hu, W.Jiang, L.Liu, S.Lan, X.Song, and C.Liu (2010).
DNA-triggered aggregation of copper, zinc superoxide dismutase in the presence of ascorbate.
  PLoS One, 5, e12328.  
19888600 K.Chen, F.J.Northington, and L.J.Martin (2010).
Inducible nitric oxide synthase is present in motor neuron mitochondria and Schwann cells and contributes to disease mechanisms in ALS mice.
  Brain Struct Funct, 214, 219-234.  
20175748 K.E.Muirhead, E.Borger, L.Aitken, S.J.Conway, and F.J.Gunn-Moore (2010).
The consequences of mitochondrial amyloid beta-peptide in Alzheimer's disease.
  Biochem J, 426, 255-270.  
19651206 L.J.Martin (2010).
The mitochondrial permeability transition pore: a molecular target for amyotrophic lateral sclerosis therapy.
  Biochim Biophys Acta, 1802, 186-197.  
21258649 L.J.Martin (2010).
Mitochondrial and Cell Death Mechanisms in Neurodegenerative Diseases.
  Pharmaceuticals (Basel), 3, 839-915.  
20001734 M.Csala, E.Margittai, and G.Bánhegyi (2010).
Redox control of endoplasmic reticulum function.
  Antioxid Redox Signal, 13, 77.  
20394052 M.Paizs, J.I.Engelhardt, Z.Katarova, and L.Siklós (2010).
Hypoglossal motor neurons display a reduced calcium increase after axotomy in mice with upregulated parvalbumin.
  J Comp Neurol, 518, 1946-1961.  
20309572 M.Rabe, A.Felbecker, S.Waibel, P.Steinbach, P.Winter, U.Müller, and A.C.Ludolph (2010).
The epidemiology of CuZn-SOD mutations in Germany: a study of 217 families.
  J Neurol, 257, 1298-1302.  
20223753 M.Wong, and L.J.Martin (2010).
Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice.
  Hum Mol Genet, 19, 2284-2302.  
20498711 R.Chia, M.H.Tattum, S.Jones, J.Collinge, E.M.Fisher, and G.S.Jackson (2010).
Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis.
  PLoS One, 5, e10627.  
19800308 A.Galaleldeen, R.W.Strange, L.J.Whitson, S.V.Antonyuk, N.Narayana, A.B.Taylor, J.P.Schuermann, S.P.Holloway, S.S.Hasnain, and P.J.Hart (2009).
Structural and biophysical properties of metal-free pathogenic SOD1 mutants A4V and G93A.
  Arch Biochem Biophys, 492, 40-47.
PDB codes: 2wko 3gzo 3gzp 3gzq
19227972 D.D.Winkler, J.P.Schuermann, X.Cao, S.P.Holloway, D.R.Borchelt, M.C.Carroll, J.B.Proescher, V.C.Culotta, and P.J.Hart (2009).
Structural and biophysical properties of the pathogenic SOD1 variant H46R/H48Q.
  Biochemistry, 48, 3436-3447.
PDB code: 3gqf
19063897 D.S.Shin, M.Didonato, D.P.Barondeau, G.L.Hura, C.Hitomi, J.A.Berglund, E.D.Getzoff, S.C.Cary, and J.A.Tainer (2009).
Superoxide dismutase from the eukaryotic thermophile Alvinella pompejana: structures, stability, mechanism, and insights into amyotrophic lateral sclerosis.
  J Mol Biol, 385, 1534-1555.
PDB codes: 3f7k 3f7l
19593442 G.T.Banks, V.Bros-Facer, H.P.Williams, R.Chia, F.Achilli, J.B.Bryson, L.Greensmith, and E.M.Fisher (2009).
Mutant glycyl-tRNA synthetase (Gars) ameliorates SOD1(G93A) motor neuron degeneration phenotype but has little affect on Loa dynein heavy chain mutant mice.
  PLoS One, 4, e6218.  
19104447 H.Sumi, S.Kato, Y.Mochimaru, H.Fujimura, M.Etoh, and S.Sakoda (2009).
Nuclear TAR DNA binding protein 43 expression in spinal cord neurons correlates with the clinical course in amyotrophic lateral sclerosis.
  J Neuropathol Exp Neurol, 68, 37-47.  
19080755 J.Andrews (2009).
Amyotrophic lateral sclerosis: clinical management and research update.
  Curr Neurol Neurosci Rep, 9, 59-68.  
19265433 J.J.Perry, A.S.Hearn, D.E.Cabelli, H.S.Nick, J.A.Tainer, and D.N.Silverman (2009).
Contribution of human manganese superoxide dismutase tyrosine 34 to structure and catalysis.
  Biochemistry, 48, 3417-3424.
PDB codes: 1zsp 1zte 1zuq 2p4k
19586921 J.M.Leitch, P.J.Yick, and V.C.Culotta (2009).
The right to choose: multiple pathways for activating copper,zinc superoxide dismutase.
  J Biol Chem, 284, 24679-24683.  
19438725 J.Zhai, A.L.Ström, R.Kilty, P.Venkatakrishnan, J.White, W.V.Everson, E.J.Smart, and H.Zhu (2009).
Proteomic characterization of lipid raft proteins in amyotrophic lateral sclerosis mouse spinal cord.
  FEBS J, 276, 3308-3323.  
19309264 K.A.Trumbull, and J.S.Beckman (2009).
A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosis.
  Antioxid Redox Signal, 11, 1627-1639.  
19184563 K.Kanekura, H.Suzuki, S.Aiso, and M.Matsuoka (2009).
ER Stress and Unfolded Protein Response in Amyotrophic Lateral Sclerosis.
  Mol Neurobiol, 39, 81-89.  
19635794 K.S.Molnar, N.M.Karabacak, J.L.Johnson, Q.Wang, A.Tiwari, L.J.Hayward, S.J.Coales, Y.Hamuro, and J.N.Agar (2009).
A common property of amyotrophic lateral sclerosis-associated variants: destabilization of the copper/zinc superoxide dismutase electrostatic loop.
  J Biol Chem, 284, 30965-30973.  
19272377 L.J.Martin, B.Gertz, Y.Pan, A.C.Price, J.D.Molkentin, and Q.Chang (2009).
The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice.
  Exp Neurol, 218, 333-346.  
19176896 M.Saeed, Y.Yang, H.X.Deng, W.Y.Hung, N.Siddique, L.Dellefave, C.Gellera, P.M.Andersen, and T.Siddique (2009).
Age and founder effect of SOD1 A4V mutation causing ALS.
  Neurology, 72, 1634-1639.  
19654524 N.Udar, S.R.Atilano, K.Small, A.B.Nesburn, and M.C.Kenney (2009).
SOD1 haplotypes in familial keratoconus.
  Cornea, 28, 902-907.  
19130895 R.Liu, A.L.Ström, J.Zhai, J.Gal, S.Bao, W.Gong, and H.Zhu (2009).
Enzymatically inactive adenylate kinase 4 interacts with mitochondrial ADP/ATP translocase.
  Int J Biochem Cell Biol, 41, 1371-1380.  
19596823 S.V.Seetharaman, M.Prudencio, C.Karch, S.P.Holloway, D.R.Borchelt, and P.J.Hart (2009).
Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis.
  Exp Biol Med (Maywood), 234, 1140-1154.  
19188595 T.Awano, G.S.Johnson, C.M.Wade, M.L.Katz, G.C.Johnson, J.F.Taylor, M.Perloski, T.Biagi, I.Baranowska, S.Long, P.A.March, N.J.Olby, G.D.Shelton, S.Khan, D.P.O'Brien, K.Lindblad-Toh, and J.R.Coates (2009).
Genome-wide association analysis reveals a SOD1 mutation in canine degenerative myelopathy that resembles amyotrophic lateral sclerosis.
  Proc Natl Acad Sci U S A, 106, 2794-2799.  
19515851 T.Nonaka, F.Kametani, T.Arai, H.Akiyama, and M.Hasegawa (2009).
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.
  Hum Mol Genet, 18, 3353-3364.  
19696882 V.Castillo, and S.Ventura (2009).
Amyloidogenic regions and interaction surfaces overlap in globular proteins related to conformational diseases.
  PLoS Comput Biol, 5, e1000476.  
19326466 W.H.Cheng (2009).
Impact of inorganic nutrients on maintenance of genomic stability.
  Environ Mol Mutagen, 50, 349-360.  
19196430 X.Li, L.Lu, D.J.Bush, X.Zhang, L.Zheng, E.A.Suswam, and P.H.King (2009).
Mutant copper-zinc superoxide dismutase associated with amyotrophic lateral sclerosis binds to adenine/uridine-rich stability elements in the vascular endothelial growth factor 3'-untranslated region.
  J Neurochem, 108, 1032-1044.  
19390823 X.Xu, Y.Zhou, S.Wei, D.Ren, M.Yang, H.Bu, M.Kang, J.Wang, and J.Feng (2009).
Molecular cloning and expression of a Cu/Zn-Containing superoxide dismutase from Thellungiella halophila.
  Mol Cells, 27, 423-428.  
18384644 A.L.Ström, J.Gal, P.Shi, E.J.Kasarskis, L.J.Hayward, and H.Zhu (2008).
Retrograde axonal transport and motor neuron disease.
  J Neurochem, 106, 495-505.  
18515363 A.L.Ström, P.Shi, F.Zhang, J.Gal, R.Kilty, L.J.Hayward, and H.Zhu (2008).
Interaction of amyotrophic lateral sclerosis (ALS)-related mutant copper-zinc superoxide dismutase with the dynein-dynactin complex contributes to inclusion formation.
  J Biol Chem, 283, 22795-22805.  
19436494 A.Nordlund, and M.Oliveberg (2008).
SOD1-associated ALS: a promising system for elucidating the origin of protein-misfolding disease.
  HFSP J, 2, 354-364.  
18424447 D.Han-Xiang, J.Hujun, F.Ronggen, Z.Hong, S.Yong, L.Erdong, H.Makito, C.D.Mauro, and S.Teepu (2008).
Molecular dissection of ALS-associated toxicity of SOD1 in transgenic mice using an exon-fusion approach.
  Hum Mol Genet, 17, 2310-2319.  
18436292 D.J.McAdoo, and P.Wu (2008).
Microdialysis in central nervous system disorders and their treatment.
  Pharmacol Biochem Behav, 90, 282-296.  
18522489 D.Trachootham, W.Lu, M.A.Ogasawara, R.D.Nilsa, and P.Huang (2008).
Redox regulation of cell survival.
  Antioxid Redox Signal, 10, 1343-1374.  
18592312 G.T.Banks, A.Kuta, A.M.Isaacs, and E.M.Fisher (2008).
TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander.
  Mamm Genome, 19, 299-305.  
18481174 J.Brettschneider, H.Mogel, V.Lehmensiek, T.Ahlert, S.Süssmuth, A.C.Ludolph, and H.Tumani (2008).
Proteome analysis of cerebrospinal fluid in amyotrophic lateral sclerosis (ALS).
  Neurochem Res, 33, 2358-2363.  
18485920 K.H.Park, and I.Vincent (2008).
Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis.
  Biochim Biophys Acta, 1782, 462-468.  
18022844 M.C.Ng, J.T.Ho, S.L.Ho, R.Lee, G.Li, T.S.Cheng, Y.Q.Song, P.W.Ho, G.C.Fong, W.Mak, K.H.Chan, L.S.Li, K.D.Luk, Y.Hu, D.B.Ramsden, and L.L.Leong (2008).
Abnormal diffusion tensor in nonsymptomatic familial amyotrophic lateral sclerosis with a causative superoxide dismutase 1 mutation.
  J Magn Reson Imaging, 27, 8.  
18370853 M.Cozzolino, A.Ferri, and M.T.Carrì (2008).
Amyotrophic lateral sclerosis: from current developments in the laboratory to clinical implications.
  Antioxid Redox Signal, 10, 405-443.  
18991862 M.Ferrucci, L.Pasquali, A.Paparelli, S.Ruggieri, and F.Fornai (2008).
Pathways of methamphetamine toxicity.
  Ann N Y Acad Sci, 1139, 177-185.  
18625408 N.Siddique, and T.Siddique (2008).
Genetics of amyotrophic lateral sclerosis.
  Phys Med Rehabil Clin N Am, 19, 429.  
18061359 S.E.Smittkamp, J.W.Brown, and J.A.Stanford (2008).
Time-course and characterization of orolingual motor deficits in B6SJL-Tg(SOD1-G93A)1Gur/J mice.
  Neuroscience, 151, 613-621.  
18026741 S.Kato (2008).
Amyotrophic lateral sclerosis models and human neuropathology: similarities and differences.
  Acta Neuropathol, 115, 97.  
18690666 W.Jiang, B.Zhang, J.Yin, L.Liu, L.Wang, and C.Liu (2008).
Polymorphism of the SOD1-DNA aggregation species can be modulated by DNA.
  Biopolymers, 89, 1154-1169.  
18378676 X.Cao, S.V.Antonyuk, S.V.Seetharaman, L.J.Whitson, A.B.Taylor, S.P.Holloway, R.W.Strange, P.A.Doucette, J.S.Valentine, A.Tiwari, L.J.Hayward, S.Padua, J.A.Cohlberg, S.S.Hasnain, and P.J.Hart (2008).
Structures of the G85R variant of SOD1 in familial amyotrophic lateral sclerosis.
  J Biol Chem, 283, 16169-16177.
PDB codes: 2vr6 2vr7 2vr8 3cqp 3cqq
18543336 Y.Ohta, T.Kamiya, M.Nagai, T.Nagata, N.Morimoto, K.Miyazaki, T.Murakami, T.Kurata, Y.Takehisa, Y.Ikeda, S.Asoh, S.Ohta, and K.Abe (2008).
Therapeutic benefits of intrathecal protein therapy in a mouse model of amyotrophic lateral sclerosis.
  J Neurosci Res, 86, 3028-3037.  
17984685 A.Ishigaki, M.Aoki, M.Nagai, H.Warita, S.Kato, M.Kato, T.Nakamura, H.Funakoshi, and Y.Itoyama (2007).
Intrathecal delivery of hepatocyte growth factor from amyotrophic lateral sclerosis onset suppresses disease progression in rat amyotrophic lateral sclerosis model.
  J Neuropathol Exp Neurol, 66, 1037-1044.  
17036243 A.Tagawa, C.F.Tan, K.Kikugawa, M.Fukase, R.Nakano, O.Onodera, M.Nishizawa, and H.Takahashi (2007).
Familial amyotrophic lateral sclerosis: a SOD1-unrelated Japanese family of bulbar type with Bunina bodies and ubiquitin-positive skein-like inclusions in lower motor neurons.
  Acta Neuropathol, 113, 205-211.  
17888947 B.R.Roberts, J.A.Tainer, E.D.Getzoff, D.A.Malencik, S.R.Anderson, V.C.Bomben, K.R.Meyers, P.A.Karplus, and J.S.Beckman (2007).
Structural characterization of zinc-deficient human superoxide dismutase and implications for ALS.
  J Mol Biol, 373, 877-890.
PDB code: 2r27
18078545 C.D.Putnam, M.Hammel, G.L.Hura, and J.A.Tainer (2007).
X-ray solution scattering (SAXS) combined with crystallography and computation: defining accurate macromolecular structures, conformations and assemblies in solution.
  Q Rev Biophys, 40, 191-285.  
17979159 C.Di Poto, P.Iadarola, A.M.Bardoni, I.Passadore, S.Giorgetti, C.Cereda, M.T.Carrì, M.Ceroni, and R.Salvini (2007).
2-DE and MALDI-TOF-MS for a comparative analysis of proteins expressed in different cellular models of amyotrophic lateral sclerosis.
  Electrophoresis, 28, 4320-4329.  
17987632 C.Di Poto, P.Iadarola, R.Salvini, I.Passadore, C.Cereda, M.Ceroni, and A.M.Bardoni (2007).
Optimizing separation efficiency of 2-DE procedures for visualization of different superoxide dismutase forms in a cellular model of amyotrophic lateral sclerosis.
  Electrophoresis, 28, 4340-4347.  
17333220 C.F.Tan, H.Eguchi, A.Tagawa, O.Onodera, T.Iwasaki, A.Tsujino, M.Nishizawa, A.Kakita, and H.Takahashi (2007).
TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation.
  Acta Neuropathol, 113, 535-542.  
17245412 C.Haass, and D.J.Selkoe (2007).
Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide.
  Nat Rev Mol Cell Biol, 8, 101-112.  
17373702 C.Neusch, M.Bähr, and C.Schneider-Gold (2007).
Glia cells in amyotrophic lateral sclerosis: new clues to understanding an old disease?
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PDB code: 1do5
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Heterodimer formation between superoxide dismutase and its copper chaperone.
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10850833 E.W.Polack, J.M.King, J.F.Cummings, H.O.Mohammed, M.Birch, and T.Cronin (2000).
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10889018 H.Liu, H.Zhu, D.K.Eggers, A.M.Nersissian, K.F.Faull, J.J.Goto, J.Ai, J.Sanders-Loehr, E.B.Gralla, and J.S.Valentine (2000).
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X-ray crystallographic and analytical ultracentrifugation analyses of truncated and full-length yeast copper chaperones for SOD (LYS7): a dimer-dimer model of LYS7-SOD association and copper delivery.
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PDB code: 1ej8
11033417 M.Bogdanov, R.H.Brown, W.Matson, R.Smart, D.Hayden, H.O'Donnell, M.Flint Beal, and M.Cudkowicz (2000).
Increased oxidative damage to DNA in ALS patients.
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Crystal structure of the Atx1 metallochaperone protein at 1.02 A resolution.
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PDB codes: 1cc7 1cc8
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Peroxynitrite: reactive, invasive and enigmatic.
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A model for the incorporation of metal from the copper chaperone CCS into Cu,Zn superoxide dismutase.
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A structure-based mechanism for copper-zinc superoxide dismutase.
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PDB codes: 1b4l 1b4t 1f18 1f1a 1f1d 1f1g 1yaz 2jcw
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Molecular mechanisms regulating motor neuron development and degeneration.
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CuZn-SOD promoter-driven expression in the Drosophila central nervous system.
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Modelling motor neuron degenerative disease.
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Solution structure of reduced monomeric Q133M2 copper, zinc superoxide dismutase (SOD). Why is SOD a dimeric enzyme?.
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PDB code: 1ba9
9585360 M.Aoki, C.L.Lin, J.D.Rothstein, B.A.Geller, B.A.Hosler, T.L.Munsat, H.R.Horvitz, and R.H.Brown (1998).
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Histochemical localization of superoxide dismutase activity in rat brain.
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Apoptosis of retrogradely degenerating neurons occurs in association with the accumulation of perikaryal mitochondria and oxidative damage to the nucleus.
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Activated alpha 2-macroglobulin reverses the immunosuppressive activity in human breast cancer cell-conditioned medium by selectively neutralizing transforming growth factor-beta in the presence of interleukin-2.
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The genetic and molecular mechanisms of motor neuron disease.
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Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis.
  Protein Sci, 7, 545-555.
PDB code: 1azv
9539776 P.Wang, H.Chen, H.Qin, S.Sankarapandi, M.W.Becher, P.C.Wong, and J.L.Zweier (1998).
Overexpression of human copper, zinc-superoxide dismutase (SOD1) prevents postischemic injury.
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Reexamination of the mechanism of hydroxyl radical adducts formed from the reaction between familial amyotrophic lateral sclerosis-associated Cu,Zn superoxide dismutase mutants and H2O2.
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Antibodies for targeted gene therapy: extracellular gene targeting and intracellular expression.
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The manganese superoxide dismutase gene of Drosophila: structure, expression, and evidence for regulation by MAP kinase.
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Molecular basis of sulfite oxidase deficiency from the structure of sulfite oxidase.
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PDB code: 1sox
9294870 C.L.Fisher, D.E.Cabelli, R.A.Hallewell, P.Beroza, T.P.Lo, E.D.Getzoff, and J.A.Tainer (1997).
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PDB code: 1fun
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Amyloid precursor protein, copper and Alzheimer's disease.
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A new type of hereditary motor and sensory neuropathy linked to chromosome 3.
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Catalase is a sink for H2O2 and is indispensable for stress defence in C3 plants.
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Amphiphilic peroxynitrite decomposition catalysts in liposomal assemblies.
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Novel G16S (GGC-AGC) mutation in the SOD-1 gene in a patient with apparently sporadic young-onset amyotrophic lateral sclerosis.
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Neurodegenerative disorders in humans: the role of glutathione in oxidative stress-mediated neuronal death.
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Clinical and molecular analysis of neurodegenerative diseases.
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Melatonin rescues dopamine neurons from cell death in tissue culture models of oxidative stress.
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A critical assessment of the evidence from XAFS and crystallography for the breakage of the imidazolate bridge during catalysis in CuZn superoxide dismutase.
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Progressive motor neuron impairment in an animal model of familial amyotrophic lateral sclerosis.
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Function of the Greek key connection analysed using circular permutants of superoxide dismutase.
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Transgenic animal models of familial amyotrophic lateral sclerosis.
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Mutagenesis of a proton linkage pathway in Escherichia coli manganese superoxide dismutase.
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Oxidative stress mediates impairment of muscle function in transgenic mice with elevated level of wild-type Cu/Zn superoxide dismutase.
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Crystallization and preliminary X-ray analysis of the monomeric Cu,Zn superoxide dismutase from Escherichia coli.
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From genes to mechanisms to therapies: lessons to be learned from neurological disorders.
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Biochemistry of cell death.
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Non-invasive early prenatal molecular diagnosis using retrieved transcervical trophoblast cells.
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Copper/zinc superoxide dismutase expression in the human central nervous system. Correlation with selective neuronal vulnerability.
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A specific superoxide dismutase mutation is on the same genetic background in sporadic and familial cases of amyotrophic lateral sclerosis.
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The roles of free radicals in amyotrophic lateral sclerosis.
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Human mitochondrial manganese superoxide dismutase polymorphic variant Ile58Thr reduces activity by destabilizing the tetrameric interface.
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PDB code: 1var
8813989 J.Wokke (1996).
Riluzole.
  Lancet, 348, 795-799.  
8841988 K.Fujita, M.Yamauchi, K.Shibayama, M.Ando, M.Honda, and Y.Nagata (1996).
Decreased cytochrome c oxidase activity but unchanged superoxide dismutase and glutathione peroxidase activities in the spinal cords of patients with amyotrophic lateral sclerosis.
  J Neurosci Res, 45, 276-281.  
8930947 L.I.Bruijn, and D.W.Cleveland (1996).
Mechanisms of selective motor neuron death in ALS: insights from transgenic mouse models of motor neuron disease.
  Neuropathol Appl Neurobiol, 22, 373-387.  
8967752 L.Siklós, J.Engelhardt, Y.Harati, R.G.Smith, F.Joó, and S.H.Appel (1996).
Ultrastructural evidence for altered calcium in motor nerve terminals in amyotropic lateral sclerosis.
  Ann Neurol, 39, 203-216.  
8650157 M.B.Yim, J.H.Kang, H.S.Yim, H.S.Kwak, P.B.Chock, and E.R.Stadtman (1996).
A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide.
  Proc Natl Acad Sci U S A, 93, 5709-5714.  
8577764 M.C.Bennett, G.W.Mlady, M.Fleshner, and G.M.Rose (1996).
Synergy between chronic corticosterone and sodium azide treatments in producing a spatial learning deficit and inhibiting cytochrome oxidase activity.
  Proc Natl Acad Sci U S A, 93, 1330-1334.  
8967745 M.E.Gurney, F.B.Cutting, P.Zhai, A.Doble, C.P.Taylor, P.K.Andrus, and E.D.Hall (1996).
Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis.
  Ann Neurol, 39, 147-157.  
8647082 M.E.Schininà, P.Carlini, F.Polticelli, F.Zappacosta, F.Bossa, and L.Calabrese (1996).
Amino acid sequence of chicken Cu, Zn-containing superoxide dismutase and identification of glutathionyl adducts at exposed cysteine residues.
  Eur J Biochem, 237, 433-439.  
8651652 M.Jackson, K.E.Morrison, A.Al-Chalabi, M.Bakker, and P.N.Leigh (1996).
Analysis of chromosome 5q13 genes in amyotrophic lateral sclerosis: homozygous NAIP deletion in a sporadic case.
  Ann Neurol, 39, 796-800.  
8937830 M.K.Lee, D.R.Borchelt, P.C.Wong, S.S.Sisodia, and D.L.Price (1996).
Transgenic models of neurodegenerative diseases.
  Curr Opin Neurobiol, 6, 651-660.  
8721614 O.I.Aruoma (1996).
Characterization of drugs as antioxidant prophylactics.
  Free Radic Biol Med, 20, 675-705.  
8610185 P.H.Tu, P.Raju, K.A.Robinson, M.E.Gurney, J.Q.Trojanowski, and V.M.Lee (1996).
Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.
  Proc Natl Acad Sci U S A, 93, 3155-3160.  
8967744 R.H.Brown (1996).
Superoxide dismutase and familial amyotrophic lateral sclerosis: new insights into mechanisms and treatments.
  Ann Neurol, 39, 145-146.  
8790408 R.M.Lebovitz, H.Zhang, H.Vogel, J.Cartwright, L.Dionne, N.Lu, S.Huang, and M.M.Matzuk (1996).
Neurodegeneration, myocardial injury, and perinatal death in mitochondrial superoxide dismutase-deficient mice.
  Proc Natl Acad Sci U S A, 93, 9782-9787.  
8651656 R.W.Orrell, J.Habgood, P.Rudge, R.J.Lane, and J.S.de Belleroche (1996).
Difficulties in distinguishing sporadic from familial amyotrophic lateral sclerosis.
  Ann Neurol, 39, 810-812.  
8973140 S.D.Brown, and J.Peters (1996).
Combining mutagenesis and genomics in the mouse--closing the phenotype gap.
  Trends Genet, 12, 433-435.  
  8887660 S.J.Lin, and V.C.Culotta (1996).
Suppression of oxidative damage by Saccharomyces cerevisiae ATX2, which encodes a manganese-trafficking protein that localizes to Golgi-like vesicles.
  Mol Cell Biol, 16, 6303-6312.  
8967746 S.Przedborski, D.Donaldson, M.Jakowec, S.J.Kish, M.Guttman, G.Rosoklija, and A.P.Hays (1996).
Brain superoxide dismutase, catalase, and glutathione peroxidase activities in amyotrophic lateral sclerosis.
  Ann Neurol, 39, 158-165.  
8901564 T.J.Lyons, H.Liu, J.J.Goto, A.Nersissian, J.A.Roe, J.A.Graden, C.Café, L.M.Ellerby, D.E.Bredesen, E.B.Gralla, and J.S.Valentine (1996).
Mutations in copper-zinc superoxide dismutase that cause amyotrophic lateral sclerosis alter the zinc binding site and the redox behavior of the protein.
  Proc Natl Acad Sci U S A, 93, 12240-12244.  
  8754854 W.Gu, and N.R.Hecht (1996).
Translation of a testis-specific Cu/Zn superoxide dismutase (SOD-1) mRNA is regulated by a 65-kilodalton protein which binds to its 5' untranslated region.
  Mol Cell Biol, 16, 4535-4543.  
8797527 X.Mu, J.He, D.W.Anderson, J.Q.Trojanowski, and J.E.Springer (1996).
Altered expression of bcl-2 and bax mRNA in amyotrophic lateral sclerosis spinal cord motor neurons.
  Ann Neurol, 40, 379-386.  
8917495 Y.Bourne, S.M.Redford, H.M.Steinman, J.R.Lepock, J.A.Tainer, and E.D.Getzoff (1996).
Novel dimeric interface and electrostatic recognition in bacterial Cu,Zn superoxide dismutase.
  Proc Natl Acad Sci U S A, 93, 12774-12779.
PDB code: 1yai
7783764 A.Eisen (1995).
Amyotrophic lateral sclerosis is a multifactorial disease.
  Muscle Nerve, 18, 741-752.  
  7856748 A.Furuta, D.L.Price, C.A.Pardo, J.C.Troncoso, Z.S.Xu, N.Taniguchi, and L.J.Martin (1995).
Localization of superoxide dismutases in Alzheimer's disease and Down's syndrome neocortex and hippocampus.
  Am J Pathol, 146, 357-367.  
  7887412 A.Pramatarova, D.A.Figlewicz, A.Krizus, F.Y.Han, I.Ceballos-Picot, A.Nicole, M.Dib, V.Meininger, R.H.Brown, and G.A.Rouleau (1995).
Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis.
  Am J Hum Genet, 56, 592-596.  
  7806049 B.G.Rosser, and G.J.Gores (1995).
Liver cell necrosis: cellular mechanisms and clinical implications.
  Gastroenterology, 108, 252-275.  
7862672 C.A.Pardo, Z.Xu, D.R.Borchelt, D.L.Price, S.S.Sisodia, and D.W.Cleveland (1995).
Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons.
  Proc Natl Acad Sci U S A, 92, 954-958.  
7896175 D.Bartlett, D.F.Church, P.L.Bounds, and W.H.Koppenol (1995).
The kinetics of the oxidation of L-ascorbic acid by peroxynitrite.
  Free Radic Biol Med, 18, 85-92.  
8526456 D.E.Bredesen (1995).
Neural apoptosis.
  Ann Neurol, 38, 839-851.  
7818241 D.R.Mosier, P.Baldelli, O.Delbono, R.G.Smith, M.E.Alexianu, S.H.Appel, and E.Stefani (1995).
Amyotrophic lateral sclerosis immunoglobulins increase Ca2+ currents in a motoneuron cell line.
  Ann Neurol, 37, 102-109.  
7567970 E.R.McCabe (1995).
Modeling Lou Gehrig's disease in the fruit fly.
  Proc Natl Acad Sci U S A, 92, 8533-8534.  
8604436 G.Powis, M.Briehl, and J.Oblong (1995).
Redox signalling and the control of cell growth and death.
  Pharmacol Ther, 68, 149-173.  
8580720 J.B.Martin (1995).
CNS genetic disorders: loss of function, gain of function, or something else?
  Curr Opin Neurobiol, 5, 669-673.  
7580159 J.E.Kraus, and J.O.McNamara (1995).
Clinical relevance of defects in signalling pathways.
  Curr Opin Neurobiol, 5, 358-366.  
8525800 J.F.Cummings, A.de Lahunta, H.O.Mohammed, T.J.Divers, B.A.Summers, B.A.Valentine, and C.A.Jackson (1995).
Endothelial lipopigment as an indicator of alpha-tocopherol deficiency in two equine neurodegenerative diseases.
  Acta Neuropathol, 90, 266-272.  
8561959 J.L.Cadet, S.F.Ali, R.B.Rothman, and C.J.Epstein (1995).
Neurotoxicity, drugs and abuse, and the CuZn-superoxide dismutase transgenic mice.
  Mol Neurobiol, 11, 155-163.  
7567977 J.P.Phillips, J.A.Tainer, E.D.Getzoff, G.L.Boulianne, K.Kirby, and A.J.Hilliker (1995).
Subunit-destabilizing mutations in Drosophila copper/zinc superoxide dismutase: neuropathology and a model of dimer dysequilibrium.
  Proc Natl Acad Sci U S A, 92, 8574-8578.  
  8592323 J.de Belleroche, R.Orrell, and A.King (1995).
Familial amyotrophic lateral sclerosis/motor neurone disease (FALS): a review of current developments.
  J Med Genet, 32, 841-847.  
8575445 L.Banci, I.Bertini, C.Y.Chiu, G.T.Mullenbach, and M.S.Viezzoli (1995).
Synthesis and characterization of a monomeric mutant Cu/Zn superoxide dismutase with partially reconstituted enzymic activity.
  Eur J Biochem, 234, 855-860.  
7556154 L.Banci, I.Bertini, M.Borsari, M.S.Viezzoli, and R.A.Hallewell (1995).
Mutation of the metal-bridging proton-donor His63 residue in human Cu, Zn superoxide dismutase. Biochemical and biophysical analysis of the His63-->Cys mutant.
  Eur J Biochem, 232, 220-225.  
7857640 L.J.Greenlund, T.L.Deckwerth, and E.M.Johnson (1995).
Superoxide dismutase delays neuronal apoptosis: a role for reactive oxygen species in programmed neuronal death.
  Neuron, 14, 303-315.  
7739642 M.A.Meyer, and N.T.Potter (1995).
Sporadic ALS and chromosome 22: evidence for a possible neurofilament gene defect.
  Muscle Nerve, 18, 536-539.  
7755363 M.Aoki, K.Abe, K.Houi, M.Ogasawara, Y.Matsubara, T.Kobayashi, S.Mochio, K.Narisawa, and Y.Itoyama (1995).
Variance of age at onset in a Japanese family with amyotrophic lateral sclerosis associated with a novel Cu/Zn superoxide dismutase mutation.
  Ann Neurol, 37, 676-679.  
7846037 M.E.Ripps, G.W.Huntley, P.R.Hof, J.H.Morrison, and J.W.Gordon (1995).
Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis.
  Proc Natl Acad Sci U S A, 92, 689-693.  
7668820 M.F.Beal (1995).
Aging, energy, and oxidative stress in neurodegenerative diseases.
  Ann Neurol, 38, 357-366.  
  7588627 M.Peled-Kamar, J.Lotem, E.Okon, L.Sachs, and Y.Groner (1995).
Thymic abnormalities and enhanced apoptosis of thymocytes and bone marrow cells in transgenic mice overexpressing Cu/Zn-superoxide dismutase: implications for Down syndrome.
  EMBO J, 14, 4985-4993.  
7797096 M.S.Paller, and J.W.Eaton (1995).
Hazards of antioxidant combinations containing superoxide dismutase.
  Free Radic Biol Med, 18, 883-890.  
7585038 N.Nakao, E.M.Frodl, H.Widner, E.Carlson, F.A.Eggerding, C.J.Epstein, and P.Brundin (1995).
Overexpressing Cu/Zn superoxide dismutase enhances survival of transplanted neurons in a rat model of Parkinson's disease.
  Nat Med, 1, 226-231.  
  8530925 P.D.Swanson (1995).
Diagnosis of inherited metabolic disorders affecting the nervous system.
  J Neurol Neurosurg Psychiatry, 59, 460-470.  
  7862131 P.J.Lapinskas, K.W.Cunningham, X.F.Liu, G.R.Fink, and V.C.Culotta (1995).
Mutations in PMR1 suppress oxidative damage in yeast cells lacking superoxide dismutase.
  Mol Cell Biol, 15, 1382-1388.  
7647793 P.M.Andersen, P.Nilsson, V.Ala-Hurula, M.L.Keränen, I.Tarvainen, T.Haltia, L.Nilsson, M.Binzer, L.Forsgren, and S.L.Marklund (1995).
Amyotrophic lateral sclerosis associated with homozygosity for an Asp90Ala mutation in CuZn-superoxide dismutase.
  Nat Genet, 10, 61-66.  
7570184 R.Berger, E.Mezey, K.P.Clancy, G.Harta, R.M.Wright, J.E.Repine, R.H.Brown, M.Brownstein, and D.Patterson (1995).
Analysis of aldehyde oxidase and xanthine dehydrogenase/oxidase as possible candidate genes for autosomal recessive familial amyotrophic lateral sclerosis.
  Somat Cell Mol Genet, 21, 121-131.  
8884889 R.D.Crosland (1995).
Action of reactive oxygen species and their antagonists on twitch tension of the rat phrenic nerve-diaphragm.
  Pharmacol Toxicol, 77, 231-237.  
7598450 R.G.Smith, and S.H.Appel (1995).
Molecular approaches to amyotrophic lateral sclerosis.
  Annu Rev Med, 46, 133-145.  
7889564 R.H.Brown (1995).
Amyotrophic lateral sclerosis: recent insights from genetics and transgenic mice.
  Cell, 80, 687-692.  
8805422 R.H.Brown (1995).
Superoxide dismutase in familial amyotrophic lateral sclerosis: models for gain of function.
  Curr Opin Neurobiol, 5, 841-846.  
  7673954 R.W.Orrell, A.W.King, D.A.Hilton, M.J.Campbell, R.J.Lane, and J.S.de Belleroche (1995).
Familial amyotrophic lateral sclerosis with a point mutation of SOD-1: intrafamilial heterogeneity of disease duration associated with neurofibrillary tangles.
  J Neurol Neurosurg Psychiatry, 59, 266-270.  
7731983 S.J.Lin, and V.C.Culotta (1995).
The ATX1 gene of Saccharomyces cerevisiae encodes a small metal homeostasis factor that protects cells against reactive oxygen toxicity.
  Proc Natl Acad Sci U S A, 92, 3784-3788.  
7668834 S.Mui, G.W.Rebeck, D.McKenna-Yasek, B.T.Hyman, and R.H.Brown (1995).
Apolipoprotein E epsilon 4 allele is not associated with earlier age at onset in amyotrophic lateral sclerosis.
  Ann Neurol, 38, 460-463.  
7708768 S.Rabizadeh, E.B.Gralla, D.R.Borchelt, R.Gwinn, J.S.Valentine, S.Sisodia, P.Wong, M.Lee, H.Hahn, and D.E.Bredesen (1995).
Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells.
  Proc Natl Acad Sci U S A, 92, 3024-3028.  
8588576 W.G.Johnson, P.R.Lucek, S.Chatkupt, Y.Furman, A.Lustenberger, and A.Lazzarini (1995).
Reduced fecundity in male ALS gene-carriers.
  Am J Med Genet, 59, 149-153.  
7531887 A.A.Boldyrev (1994).
Carnosine and free-radical defence mechanisms.
  Trends Neurosci, 17, 468.  
7920663 A.Hentati, K.Bejaoui, M.A.Pericak-Vance, F.Hentati, M.C.Speer, W.Y.Hung, D.A.Figlewicz, J.Haines, J.Rimmler, and C.Ben Hamida (1994).
Linkage of recessive familial amyotrophic lateral sclerosis to chromosome 2q33-q35.
  Nat Genet, 7, 425-428.  
8054976 B.Levinson, C.Vulpe, B.Elder, C.Martin, F.Verley, S.Packman, and J.Gitschier (1994).
The mottled gene is the mouse homologue of the Menkes disease gene.
  Nat Genet, 6, 369-373.  
  8019310 C.J.Woodall, M.H.Riding, D.I.Graham, and G.B.Clements (1994).
Sequences specific for enterovirus detected in spinal cord from patients with motor neurone disease.
  BMJ, 308, 1541-1543.  
8066083 C.L.Fisher, D.E.Cabelli, J.A.Tainer, R.A.Hallewell, and E.D.Getzoff (1994).
The role of arginine 143 in the electrostatics and mechanism of Cu,Zn superoxide dismutase: computational and experimental evaluation by mutational analysis.
  Proteins, 19, 24-34.  
8022792 C.M.Troy, and M.L.Shelanski (1994).
Down-regulation of copper/zinc superoxide dismutase causes apoptotic death in PC12 neuronal cells.
  Proc Natl Acad Sci U S A, 91, 6384-6387.  
7937915 C.R.Nishida, E.B.Gralla, and J.S.Valentine (1994).
Characterization of three yeast copper-zinc superoxide dismutase mutants analogous to those coded for in familial amyotrophic lateral sclerosis.
  Proc Natl Acad Sci U S A, 91, 9906-9910.  
7523689 D.Nagy, T.Kato, and P.D.Kushner (1994).
Reactive astrocytes are widespread in the cortical gray matter of amyotrophic lateral sclerosis.
  J Neurosci Res, 38, 336-347.  
8058797 D.R.Borchelt, M.K.Lee, H.S.Slunt, M.Guarnieri, Z.S.Xu, P.C.Wong, R.H.Brown, D.L.Price, S.S.Sisodia, and D.W.Cleveland (1994).
Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity.
  Proc Natl Acad Sci U S A, 91, 8292-8296.  
  8300230 E.R.James, D.C.McLean, and F.Perler (1994).
Molecular cloning of an Onchocerca volvulus extracellular Cu-Zn superoxide dismutase.
  Infect Immun, 62, 713-716.  
7982630 H.R.Warner (1994).
Superoxide dismutase, aging, and degenerative disease.
  Free Radic Biol Med, 17, 249-258.  
7985500 H.Takahashi, T.Makifuchi, R.Nakano, S.Sato, T.Inuzuka, K.Sakimura, M.Mishina, Y.Honma, S.Tsuji, and F.Ikuta (1994).
Familial amyotrophic lateral sclerosis with a mutation in the Cu/Zn superoxide dismutase gene.
  Acta Neuropathol, 88, 185-188.  
7910402 J.D.Rothstein, L.A.Bristol, B.Hosler, R.H.Brown, and R.W.Kuncl (1994).
Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons.
  Proc Natl Acad Sci U S A, 91, 4155-4159.  
7911198 J.Kawamata, H.Hasegawa, S.Shimohama, J.Kimura, S.Tanaka, and K.Ueda (1994).
Leu106-->Val (CTC-->GTC) mutation of superoxide dismutase-1 gene in patient with familial amyotrophic lateral sclerosis in Japan.
  Lancet, 343, 1501.  
  7977375 K.C.Wilhelmsen, T.Lynch, E.Pavlou, M.Higgins, and T.G.Nygaard (1994).
Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21-22.
  Am J Hum Genet, 55, 1159-1165.  
  7992831 M.C.Dal Canto, and M.E.Gurney (1994).
Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis.
  Am J Pathol, 145, 1271-1279.  
7998770 M.E.Alexianu, B.K.Ho, A.H.Mohamed, V.La Bella, R.G.Smith, and S.H.Appel (1994).
The role of calcium-binding proteins in selective motoneuron vulnerability in amyotrophic lateral sclerosis.
  Ann Neurol, 36, 846-858.  
7700446 M.F.Beal (1994).
Energy, oxidative damage, and Alzheimer's disease: clues to the underlying puzzle.
  Neurobiol Aging, 15, S171-S174.  
7874490 M.Sendtner, and H.Thoenen (1994).
Neurodegenerative disease. Oxidative stress and motorneuron disease.
  Curr Biol, 4, 1036-1039.  
  7943166 N.K.Gonatas (1994).
Rous-Whipple Award Lecture. Contributions to the physiology and pathology of the Golgi apparatus.
  Am J Pathol, 145, 751-761.  
7972157 P.H.Evans (1994).
Nutrient and toxin interactions in neurodegenerative disease.
  Proc Nutr Soc, 53, 431-442.  
7808585 R.Lathe, and R.G.Morris (1994).
Analysing brain function and dysfunction in transgenic animals.
  Neuropathol Appl Neurobiol, 20, 350-358.  
7761349 R.R.Bowers, J.Lujan, A.Biboso, S.Kridel, and C.Varkey (1994).
Premature avian melanocyte death due to low antioxidant levels of protection: fowl model for vitiligo.
  Pigment Cell Res, 7, 409-418.  
7996955 R.W.Orrell, and J.S.deBelleroche (1994).
Superoxide dismutase and ALS.
  Lancet, 344, 1651-1652.  
  8044087 R.W.Orrell, R.J.Lane, and R.J.Guiloff (1994).
Recent developments in the drug treatment of motor neurone disease.
  BMJ, 309, 140-141.  
8038390 S.T.Ferreira, L.Stella, and E.Gratton (1994).
Conformational dynamics of bovine Cu, Zn superoxide dismutase revealed by time-resolved fluorescence spectroscopy of the single tyrosine residue.
  Biophys J, 66, 1185-1196.  
7936071 S.Zeman, C.Lloyd, B.Meldrum, and P.N.Leigh (1994).
Excitatory amino acids, free radicals and the pathogenesis of motor neuron disease.
  Neuropathol Appl Neurobiol, 20, 219-231.  
7917302 T.Tsuda, S.Munthasser, P.E.Fraser, M.E.Percy, I.Rainero, G.Vaula, L.Pinessi, L.Bergamini, G.Vignocchi, and D.R.McLachlan (1994).
Analysis of the functional effects of a mutation in SOD1 associated with familial amyotrophic lateral sclerosis.
  Neuron, 13, 727-736.  
  7935419 X.F.Liu, and V.C.Culotta (1994).
The requirement for yeast superoxide dismutase is bypassed through mutations in BSD2, a novel metal homeostasis gene.
  Mol Cell Biol, 14, 7037-7045.  
  8203467 Z.Mourelatos, A.Hirano, A.C.Rosenquist, and N.K.Gonatas (1994).
Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis (ALS). Clinical studies in ALS of Guam and experimental studies in deafferented neurons and in beta,beta'-iminodipropionitrile axonopathy.
  Am J Pathol, 144, 1288-1300.  
8245985 A.C.Bowling, J.B.Schulz, R.H.Brown, and M.F.Beal (1993).
Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis.
  J Neurochem, 61, 2322-2325.  
7507613 C.W.Olanow (1993).
A radical hypothesis for neurodegeneration.
  Trends Neurosci, 16, 439-444.  
8298637 M.Aoki, M.Ogasawara, Y.Matsubara, K.Narisawa, S.Nakamura, Y.Itoyama, and K.Abe (1993).
Mild ALS in Japan associated with novel SOD mutation.
  Nat Genet, 5, 323-324.  
The most recent references are shown first. Citation data come partly from CiteXplore and partly from an automated harvesting procedure. Note that this is likely to be only a partial list as not all journals are covered by either method. However, we are continually building up the citation data so more and more references will be included with time. Where a reference describes a PDB structure, the PDB codes are shown on the right.