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Superoxide dismutase-1 and other proteins in inclusions from transgenic amyotrophic lateral sclerosis model mice
Umeå University, Faculty of Medicine, Department of Medical Biosciences, Clinical chemistry.
Umeå University, Faculty of Medicine, Department of Medical Biosciences, Pathology. (Thomas Brännström)
Umeå University, Faculty of Medicine, Department of Medical Biosciences, Pathology.
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2010 (English)In: Journal of Neurochemistry, ISSN 0022-3042, E-ISSN 1471-4159, Vol. 114, no 2, 408-418 p.Article in journal (Refereed) Published
Abstract [en]

Mutant superoxide dismutase-1 (SOD1) causes amyotrophic lateral sclerosis (ALS) through a cytotoxic mechanism of unknown nature. A hallmark in ALS patients and transgenic mouse models carrying human SOD1 (hSOD1) mutations are hSOD1-immunoreactive inclusions in spinal cord ventral horns. The hSOD1 inclusions may block essential cellular functions or cause toxicity through sequestering of other proteins. Inclusions from four different transgenic mouse models were examined after density gradient ultracentrifugation. The inclusions are complex structures with heterogeneous densities and are disrupted by detergents. The aggregated hSOD1 was mainly composed of subunits that lacked the native stabilizing intra-subunit disulfide bond. A proportion of subunits formed hSOD1 oligomers or was bound to other proteins through disulfide bonds. Dense inclusions could be isolated and the protein composition was analyzed using proteomic techniques. Mutant hSOD1 accounted for half of the protein. Ten other proteins were identified. Two were cytoplasmic chaperones, four were cytoskeletal proteins, and 4 were proteins that normally reside in the endoplasmic reticulum (ER). The presence of ER proteins in inclusions containing the primarily cytosolic hSOD1 further supports the notion that ER stress is involved in ALS.

Place, publisher, year, edition, pages
2010. Vol. 114, no 2, 408-418 p.
Keyword [en]
amyotrophic lateral sclerosis;endoplasmic reticulum;inclusion;proteomics;superoxide dismutase-1;transgenic mice
National Category
Medical and Health Sciences
Research subject
Medicine
Identifiers
URN: urn:nbn:se:umu:diva-42155DOI: 10.1111/j.1471-4159.2010.06753.xISI: 000279077900007PubMedID: 20412382OAI: oai:DiVA.org:umu-42155DiVA: diva2:408773
Available from: 2011-04-06 Created: 2011-04-06 Last updated: 2017-12-11Bibliographically approved

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Bergemalm, DanielForsberg, KarinGraffmo, Karin SAndersen, Peter MBrännström, ThomasMarklund, Stefan L
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