ArticleThe Journal of biological chemistry2010
Nonamyloid aggregates arising from mature copper/zinc superoxide dismutases resemble those observed in amyotrophic lateral sclerosis.
Article in The Journal of biological chemistry, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 58 citations in OpenAlex.
- Structural insights into SOD1: from in silico and molecular dynamics to experimental analyses of ALS-associated E49K and R115G mutants.Frontiers in molecular biosciences · 2025Article
- Implications of ALS-Associated Mutations on Biochemical and Biophysical Features of hSOD1 and Aggregation Formation.Biochemical genetics · 2024Article
- Conformational dynamics of superoxide dismutase (SOD1) in osmolytes: a molecular dynamics simulation study.RSC advances · 2020Article
- Mutations in Superoxide Dismutase 1 (Sod1) Linked to Familial Amyotrophic Lateral Sclerosis Can Disrupt High-Affinity Zinc-Binding Promoted by the Copper Chaperone for Sod1 (Ccs).Molecules (Basel, Switzerland) · 2020Article
- Tryptophan residue 32 in human Cu-Zn superoxide dismutase modulates prion-like propagation and strain selection.PloS one · 2020Article
- Pull-Down of Metalloproteins in Their Native States by Using Desthiobiotin-Based Probes.Chembiochem : a European journal of chemical biology · 2019Article
- Experimental Mutations in Superoxide Dismutase 1 Provide Insight into Potential Mechanisms Involved in Aberrant Aggregation in Familial Amyotrophic Lateral Sclerosis.G3 (Bethesda, Md.) · 2019Article
- Cholesterol secosterol aldehyde adduction and aggregation of Cu,Zn-superoxide dismutase: Potential implications in ALS.Redox biology · 2018Article
- Partially native intermediates mediate misfolding of SOD1 in single-molecule folding trajectories.Nature communications · 2017Article
- Assay Development for High Content Quantification of Sod1 Mutant Protein Aggregate Formation in Living Cells.Journal of visualized experiments : JoVE · 2017Article
- Comparison of serum Concentration of Se, Pb, Mg, Cu, Zn, between MS patients and healthy controls.Electronic physician · 2016Article
- ALS-linked misfolded SOD1 species have divergent impacts on mitochondria.Acta neuropathologica communications · 2016Article
- SOD1 aggregation in ALS mice shows simplistic test tube behavior.Proceedings of the National Academy of Sciences of the United States of America · 2015Article
- Article
- Oxidation of the tryptophan 32 residue of human superoxide dismutase 1 caused by its bicarbonate-dependent peroxidase activity triggers the non-amyloid aggregation of the enzyme.The Journal of biological chemistry · 2014Article
- Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes.Proceedings of the National Academy of Sciences of the United States of America · 2014Article
- Prognostic role of "prion-like propagation" in SOD1-linked familial ALS: an alternative view.Frontiers in cellular neuroscience · 2014Article
- 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 & selection : PEDS · 2013Article
- Cupric ions induce the oxidation and trigger the aggregation of human superoxide dismutase 1.PloS one · 2013Article
- Fibrillation precursor of superoxide dismutase 1 revealed by gradual tuning of the protein-folding equilibrium.Proceedings of the National Academy of Sciences of the United States of America · 2012Article
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Authors and funding
10 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Protein aggregation is a hallmark of many diseases, including amyotrophic lateral sclerosis (ALS) where aggregation of copper/zinc superoxide dismutase (SOD1) is implicated in pathogenesis. We report here that fully metallated (holo) SOD1 under physiologically relevant solution conditions can undergo changes in metallation and/or dimerization over time and form aggregates that do not exhibit classical characteristics of amyloid. The relevance of the observed aggregation to disease is demonstrated by structural and tinctorial analyses, including the novel observation of binding of an anti-SOD1 antibody that specifically recognizes aggregates in ALS patients and mice models. ALS-associated SOD1 mutations can promote aggregation but are not essential. The SOD1 aggregation is characterized by a lag phase, which is diminished by self- or cross-seeding and by heterogeneous nucleation. We interpret these findings in terms of an expanded aggregation mechanism consistent with other in vitro and in vivo findings that point to multiple pathways for the formation of toxic aggregates by different forms of SOD1.
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