Human Cu/Zn Superoxide Dismutase (SOD1) Overexpression in Mice Causes Mitochondrial Vacuolization, Axonal Degeneration, and Premature Motoneuron Death and Accelerates Motoneuron Disease in Mice Expressing a Familial Amyotrophic Lateral Sclerosis Mutant SOD1

Human Cu/Zn Superoxide Dismutase (SOD1) Overexpression in Mice Causes Mitochondrial Vacuolization, Axonal Degeneration, and Premature Motoneuron Death and Accelerates Motoneuron Disease in Mice Expressing a Familial Amyotrophic Lateral Sclerosis Mutant SOD1

Cytosolic Cu/Zn superoxide dismutase (SOD1) is a ubiquitous small cytosolic metalloenzyme that catalyzes the conversion of superoxide anion to hydrogen peroxide (H2O2). Mutations in the SOD1 gene cause a familial form of amyotrophic lateral sclerosis (fALS). The mechanism by which mutant SOD1s cause...

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Bibliographic Details
Main Authors: Dick Jaarsma, Elize D. Haasdijk, J.A.C. Grashorn, Richard Hawkins, Wim van Duijn, Hein W. Verspaget, Jacqueline London, Jan C. Holstege
Format: Article
Language:English
Published: Elsevier 2000-12-01
Series:Neurobiology of Disease
Subjects:
oxidative stress
amyotrophic lateral sclerosis
electron microscopy
spinal cord
neurodegenerative disease
aging.
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996100902997

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http://www.sciencedirect.com/science/article/pii/S0969996100902997

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