BDH1 and amyotrophic lateral sclerosis: Given that ALS motor neurons display a strongly reduced expression of two enzymes involved in ketone bodies formation and utilization, namely 3-hydroxybutyrate dehydrogenase 1 (BDH1) and 3-oxoacid-CoA transferase 1 (OXCT1), one could hypothesize that the acetoacetyl-CoA could be used for cholesterol synthesis by the mevalonate pathway.