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Prion-like Properties of Misfolded Cu/Zn-superoxide Dismutase in Amyotrophic Lateral Sclerosis: Update and Perspectives
Umeå University, Faculty of Medicine.ORCID iD: 0000-0002-3270-2854
2019 (English)In: Yakugaku zasshi, ISSN 0031-6903, E-ISSN 1347-5231, Vol. 139, no 7, p. 1015-1019Article, review/survey (Refereed) Published
Abstract [en]

Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease that is characterized by the loss of motor neurons, which results in progressive muscle atrophy. The pathology spreads from the initial site of onset to contiguous anatomic regions. Mutations in the gene encoding Cu/Zn-superoxide dismutase (SOD1) have been identified in a dominantly inherited form of ALS (ALS-SOD1). A major hallmark of ALS-SOD1 is the abnormal accumulation of conformationally aberrant SOD1 protein (i.e., misfolded SOD1) within motor neurons. Emerging experimental evidence has suggested that misfolded proteins associated with neurodegenerative diseases exhibit prion-like properties, i.e., misfolded proteins act as conformational templates that convert normal proteins into a pathogenic form. Possibly as a result of this prion-like self-propagation property, misfolded forms of pathological proteins are considered to accumulate in the central nervous system and cause neurodegeneration. In this article, we review recent evidence for the role of prion-like mechanisms in ALS-SOD1. In particular, we discuss the propensity of misfolded SOD1 to act as a pathological seed, spread between cells, and propagate neuroanatomically.

Place, publisher, year, edition, pages
Pharmaceutical Society of Japan , 2019. Vol. 139, no 7, p. 1015-1019
Keywords [en]
amyotrophic lateral sclerosis, superoxide dismutase-1, prion-like propagation
National Category
Neurosciences Neurology
Identifiers
URN: urn:nbn:se:umu:diva-161704DOI: 10.1248/yakushi.18-00165-5ISI: 000473562600009PubMedID: 31257248OAI: oai:DiVA.org:umu-161704DiVA, id: diva2:1340431
Available from: 2019-08-05 Created: 2019-08-05 Last updated: 2019-08-05Bibliographically approved

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Marklund, Stefan L.

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