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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Autores principales:	Simote T. Foliaki, Brent Race, Katie Williams, Chase Baune, Bradley R. Groveman, Cathryn L. Haigh
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/fd6687d15ac742fca4e0aa6fba46e13b
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spelling	oai:doaj.org-article:fd6687d15ac742fca4e0aa6fba46e13b2021-11-11T07:14:42ZReduced SOD2 expression does not influence prion disease course or pathology in mice1932-6203https://doaj.org/article/fd6687d15ac742fca4e0aa6fba46e13b2021-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8568125/?tool=EBIhttps://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 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	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
description	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/fd6687d15ac742fca4e0aa6fba46e13b
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Reduced SOD2 expression does not influence prion disease course or pathology in mice

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