Neurodegeneration and early lethality in superoxide dismutase 2-deficient mice: A comprehensive analysis of the central and peripheral nervous systems

Highlights ► Cre-lox under the nestin promoter was used to knockout SOD2 in the nervous system. ► SOD2 knockout in the nervous system was perinatally lethal due to severe seizures. ► Spongiform pathology and mitochondrial damage are seen in the central nervous system. ► Knockout of SOD2 was incomple...

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Bibliographic Details
Published in:Neuroscience 2012-06, Vol.212, p.201-213
Main Authors: Oh, S.S, Sullivan, K.A, Wilkinson, J.E, Backus, C, Hayes, J.M, Sakowski, S.A, Feldman, E.L
Format: Article
Language:English
Subjects:
Animals
Biological and medical sciences
BKS.db/db mice
Brain
Central nervous system
Central Nervous System - enzymology
Central Nervous System - pathology
Diabetes mellitus
Diabetes Mellitus, Type 2 - metabolism
Diabetes Mellitus, Type 2 - pathology
Diabetes. Impaired glucose tolerance
Diabetic Nephropathies - enzymology
Diabetic Nephropathies - genetics
Diabetic Nephropathies - pathology
diabetic neuropathy
Disease Models, Animal
Dorsal root ganglia
Endocrine pancreas. Apud cells (diseases)
Endocrinopathies
Etiopathogenesis. Screening. Investigations. Target tissue resistance
Female
Fundamental and applied biological sciences. Psychology
gait
Ganglia, Spinal - enzymology
Ganglia, Spinal - pathology
Genes, Lethal
Lethality
Male
Medical sciences
Mice
Mice, Knockout
Mice, Transgenic
Mitochondria
Myelin
Nerve Degeneration - enzymology
Nerve Degeneration - genetics
Nerve Degeneration - pathology
nervous system
Nestin
Neurodegeneration
Neurology
Neuropathy
Oxidative stress
Peripheral nerves
Peripheral nervous system
Peripheral Nervous System - enzymology
Peripheral Nervous System - pathology
Promoters
ROS
Seizures
SOD2
Spinal cord
spongiform encephalopathy
Superoxide dismutase
Superoxide Dismutase - deficiency
Superoxide Dismutase - genetics
Superoxide Dismutase - metabolism
Vertebrates: nervous system and sense organs
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Summary:Highlights ► Cre-lox under the nestin promoter was used to knockout SOD2 in the nervous system. ► SOD2 knockout in the nervous system was perinatally lethal due to severe seizures. ► Spongiform pathology and mitochondrial damage are seen in the central nervous system. ► Knockout of SOD2 was incomplete in the peripheral nervous system.
ISSN:0306-4522
1873-7544
DOI:10.1016/j.neuroscience.2012.03.026