Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase.
basic_science · Level V
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- Record sourced from PubMed, PMID 9689131.
- Also identified by PMC identifier 21389.
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Abstract
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.
Medical subject headings
- Amyotrophic Lateral Sclerosis
- Gene Expression Regulation, Enzymologic
- Neurofilament Proteins
- Superoxide Dismutase