Inactivation of liver X receptor β leads to adult-onset motor neuron degeneration in male mice

Sandra Andersson, Nina Gustafsson, Margaret Warner, Jan Åke Gustafsson

Research output: Contribution to journalArticlepeer-review

129 Scopus citations

Abstract

Male mice with inactivated liver X receptor (LXR) β suffer from adult-onset motor neuron degeneration. By 7 months of age, motor coordination is impaired, and this condition is associated with lipid accumulation and loss of motor neurons in the spinal cord, together with axonal atrophy and astrogliosis. Several of these features are reminiscent of the neuropathological signs of chronic motor neuron disease such as amyotrophic lateral sclerosis. Because the LXRs are important for cholesterol and lipid metabolism, we speculate that absence of LXRβ leads to pathological accumulation of sterols and lipids that may themselves be neurotoxic or may modulate intracellular pathways and thereby predispose motor neurons to degeneration.

Original languageEnglish (US)
Pages (from-to)3857-3862
Number of pages6
JournalProceedings of the National Academy of Sciences of the United States of America
Volume102
Issue number10
DOIs
StatePublished - Mar 8 2005

Keywords

  • Amyotrophic lateral sclerosis
  • Spinal cord

ASJC Scopus subject areas

  • Genetics
  • General

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