Reduced SOD2 expression does not influence prion disease course or pathology in mice.
Prion diseases are progressive, neurodegenerative diseases affecting humans and animals. Also known as the transmissible spongiform encephalopathies, for the hallmark spongiform change seen in the brain, these diseases manifest increased oxidative damage early in disease and changes in antioxidant e...
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2021
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oai:doaj.org-article:23606fe40de04f528270e8cb9d2e7f102021-12-02T20:04:18ZReduced SOD2 expression does not influence prion disease course or pathology in mice.1932-620310.1371/journal.pone.0259597https://doaj.org/article/23606fe40de04f528270e8cb9d2e7f102021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0259597https://doaj.org/toc/1932-6203Prion diseases are progressive, neurodegenerative diseases affecting humans and animals. Also known as the transmissible spongiform encephalopathies, for the hallmark spongiform change seen in the brain, these diseases manifest increased oxidative damage early in disease and changes in antioxidant enzymes in terminal brain tissue. Superoxide dismutase 2 (SOD2) is an antioxidant enzyme that is critical for life. SOD2 knock-out mice can only be kept alive for several weeks post-birth and only with antioxidant therapy. However, this results in the development of a spongiform encephalopathy. Consequently, we hypothesized that reduced levels of SOD2 may accelerate prion disease progression and play a critical role in the formation of spongiform change. Using SOD2 heterozygous knock-out and litter mate wild-type controls, we examined neuronal long-term potentiation, disease duration, pathology, and degree of spongiform change in mice infected with three strains of mouse adapted scrapie. No influence of the reduced SOD2 expression was observed in any parameter measured for any strain. We conclude that changes relating to SOD2 during prion disease are most likely secondary to the disease processes causing toxicity and do not influence the development of spongiform pathology.Simote T FoliakiBrent RaceKatie WilliamsChase BauneBradley R GrovemanCathryn L HaighPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 11, p e0259597 (2021) |
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Medicine R Science Q Simote T Foliaki Brent Race Katie Williams Chase Baune Bradley R Groveman Cathryn L Haigh Reduced SOD2 expression does not influence prion disease course or pathology in mice. |
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Prion diseases are progressive, neurodegenerative diseases affecting humans and animals. Also known as the transmissible spongiform encephalopathies, for the hallmark spongiform change seen in the brain, these diseases manifest increased oxidative damage early in disease and changes in antioxidant enzymes in terminal brain tissue. Superoxide dismutase 2 (SOD2) is an antioxidant enzyme that is critical for life. SOD2 knock-out mice can only be kept alive for several weeks post-birth and only with antioxidant therapy. However, this results in the development of a spongiform encephalopathy. Consequently, we hypothesized that reduced levels of SOD2 may accelerate prion disease progression and play a critical role in the formation of spongiform change. Using SOD2 heterozygous knock-out and litter mate wild-type controls, we examined neuronal long-term potentiation, disease duration, pathology, and degree of spongiform change in mice infected with three strains of mouse adapted scrapie. No influence of the reduced SOD2 expression was observed in any parameter measured for any strain. We conclude that changes relating to SOD2 during prion disease are most likely secondary to the disease processes causing toxicity and do not influence the development of spongiform pathology. |
format |
article |
author |
Simote T Foliaki Brent Race Katie Williams Chase Baune Bradley R Groveman Cathryn L Haigh |
author_facet |
Simote T Foliaki Brent Race Katie Williams Chase Baune Bradley R Groveman Cathryn L Haigh |
author_sort |
Simote T Foliaki |
title |
Reduced SOD2 expression does not influence prion disease course or pathology in mice. |
title_short |
Reduced SOD2 expression does not influence prion disease course or pathology in mice. |
title_full |
Reduced SOD2 expression does not influence prion disease course or pathology in mice. |
title_fullStr |
Reduced SOD2 expression does not influence prion disease course or pathology in mice. |
title_full_unstemmed |
Reduced SOD2 expression does not influence prion disease course or pathology in mice. |
title_sort |
reduced sod2 expression does not influence prion disease course or pathology in mice. |
publisher |
Public Library of Science (PLoS) |
publishDate |
2021 |
url |
https://doaj.org/article/23606fe40de04f528270e8cb9d2e7f10 |
work_keys_str_mv |
AT simotetfoliaki reducedsod2expressiondoesnotinfluencepriondiseasecourseorpathologyinmice AT brentrace reducedsod2expressiondoesnotinfluencepriondiseasecourseorpathologyinmice AT katiewilliams reducedsod2expressiondoesnotinfluencepriondiseasecourseorpathologyinmice AT chasebaune reducedsod2expressiondoesnotinfluencepriondiseasecourseorpathologyinmice AT bradleyrgroveman reducedsod2expressiondoesnotinfluencepriondiseasecourseorpathologyinmice AT cathrynlhaigh reducedsod2expressiondoesnotinfluencepriondiseasecourseorpathologyinmice |
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