A lipid-specific metabolic abnormality in ALS is suggested by our results, together with studies showing that a high-fat diet may alter the CNS pathology (i.e., death of motor neurons) [8] and that inactivation of the LXRβ gene, which participates in cholesterol transport from glial cells to neurons in the CNS, caused motor neuron degeneration in mice [21], as well as by evidence that the CNS, as well as the skeletal muscles, may contribute to the high lipid demand which might be associated with ALS pathogenesis [7]. Here, NR1H2 is linked to amyotrophic lateral sclerosis.