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SOD1 aggregation in ALS mice shows simplistic test tube behavior
Stockholm University, Faculty of Science, Department of Biochemistry and Biophysics.
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Number of Authors: 6
2015 (English)In: Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, E-ISSN 1091-6490, Vol. 112, no 32, 9878-9883 p.Article in journal (Refereed) Published
Abstract [en]

A longstanding challenge in studies of neurodegenerative disease has been that the pathologic protein aggregates in live tissue are not amenable to structural and kinetic analysis by conventional methods. The situation is put in focus by the current progress in demarcating protein aggregation in vitro, exposing new mechanistic details that are now calling for quantitative in vivo comparison. In this study, we bridge this gap by presenting a direct comparison of the aggregation kinetics of the ALS-associated protein superoxide dismutase 1 (SOD1) in vitro and in transgenic mice. The results based on tissue sampling by quantitative antibody assays show that the SOD1 fibrillation kinetics in vitro mirror with remarkable accuracy the spinal cord aggregate buildup and disease progression in transgenic mice. This similarity between in vitro and in vivo data suggests that, despite the complexity of live tissue, SOD1 aggregation follows robust and simplistic rules, providing new mechanistic insights into the ALS pathology and organism-level manifestation of protein aggregation phenomena in general.

Place, publisher, year, edition, pages
2015. Vol. 112, no 32, 9878-9883 p.
Keyword [en]
superoxide dismutase 1, aggregation, transgenic mice, aggregation kinetics
National Category
Biological Sciences Medical Biotechnology (with a focus on Cell Biology (including Stem Cell Biology), Molecular Biology, Microbiology, Biochemistry or Biopharmacy)
Identifiers
URN: urn:nbn:se:su:diva-120688DOI: 10.1073/pnas.1503328112ISI: 000359285100046OAI: oai:DiVA.org:su-120688DiVA: diva2:855583
Available from: 2015-09-21 Created: 2015-09-15 Last updated: 2017-12-04Bibliographically approved

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Lang, LisaDanielsson, JensOliveberg, Mikael
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