Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase

Sébastien Couillard-Després, Qinzhang Zhu, Philip C. Wong, Donald L. Price, Don W. Cleveland, Jean Pierre Julien

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)9626-9630
Number of pages5
JournalProceedings of the National Academy of Sciences of the United States of America
Volume95
Issue number16
DOIs
StatePublished - Aug 4 1998

Keywords

  • Amyotrophic lateral sclerosis
  • Intermediate filaments
  • Transgenic mice

ASJC Scopus subject areas

  • General

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