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Forschungsdatenbank PMU-SQQUID

Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase.
Couillard-Després, S; Zhu, Q; Wong, PC; Price, DL; Cleveland, DW; Julien, JP;
Proc Natl Acad Sci U S A. 1998; 95(16): 9626-9630.
Originalarbeiten (Zeitschrift)

PMU-Autor/inn/en

Couillard-Després Sébastien

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.


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


Find related publications in this database (Keywords)

amyotrophic lateral sclerosis
transgenic mice
intermediate filaments