TY - JOUR
T1 - Inactivation of liver X receptor β leads to adult-onset motor neuron degeneration in male mice
AU - Andersson, Sandra
AU - Gustafsson, Nina
AU - Warner, Margaret
AU - Gustafsson, Jan Åke
PY - 2005/3/8
Y1 - 2005/3/8
N2 - 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.
AB - 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.
KW - Amyotrophic lateral sclerosis
KW - Spinal cord
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U2 - 10.1073/pnas.0500634102
DO - 10.1073/pnas.0500634102
M3 - Article
C2 - 15738425
AN - SCOPUS:14844359386
VL - 102
SP - 3857
EP - 3862
JO - Proceedings of the National Academy of Sciences of the United States of America
JF - Proceedings of the National Academy of Sciences of the United States of America
SN - 0027-8424
IS - 10
ER -