PMU-Autor/inn/en
Couillard-Després SébastienAbstract
To investigate the role of neurofilaments in motor neuron disease caused by superoxide dismutase (SOD1) mutations, transgenic mice expressing a amyotrophic lateral sclerosis-linked SOD1 mutant (SOD1(G37R)) were mated with transgenic mice expressing human neurofilament heavy (NF-H) subunits. Unexpectedly, expression of human NF-H transgenes increased by up to 65%, the mean lifespan of SOD1(G37R) mice. Microscopic examination corroborated the protective effect of NF-H protein against SOD1 toxicity. Although massive neurodegeneration occurred in 1-yr-old mice expressing SOD1(G37R) alone, spinal root axons and motor neurons were remarkably spared in doubly SOD1(G37R);NF-H-transgenic littermates.
Useful keywords (using NLM MeSH Indexing)
Amyotrophic Lateral Sclerosis/genetics*
Amyotrophic Lateral Sclerosis/pathology
Animals
Axons/pathology
Cell Death
Gene Expression Regulation, Enzymologic/genetics*
Humans
Mice
Mice, Inbred C57BL
Mice, Transgenic
Mutation
Neurofilament Proteins/genetics*
Superoxide Dismutase/genetics*
Superoxide Dismutase/metabolism
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amyotrophic lateral sclerosis