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Article

Mutant SOD1 aggregates formed in vitro and in cultured cells are polymorphic and differ from those arising in the CNS.

Journal of neurochemistry - 1 Jan 2023

Nordström Ulrika, Lang Lisa, Ekhtiari Bidhendi Elaheh, Zetterström Per, Oliveberg Mikael, Danielsson Jens, Andersen Peter M, Marklund Stefan L

Abstract excerpt

Mutations in the human Superoxide dismutase 1 (hSOD1) gene are well-established cause of the motor neuron disease ALS. Patients and transgenic (Tg) ALS model mice carrying mutant variants develop hSOD1 aggregates in the CNS. We have identified two hSOD1 aggregate strains, which both transmit spreading template-directed aggregation and premature fatal paralysis when inoculated into adult transgenic mice. This...

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Identifiers

PMID: 36326589
DOI: 10.1111/jnc.15718
PMCID: PMC10099669

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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