Forschungsdatenbank PMU-SQQUID

PMU-Autor/inn/en

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)