TARDBP and amyotrophic lateral sclerosis: A recent study in a Drosophila model of TDP-43-associated ALS revealed a significant increase in long-chain fatty acid carnitine conjugates such as myristoylcarnitine, palmitoylcarnitine, oleoylcarnitine and linoleoylcarnitine, in the context of both mutant and wild-type TDP-43 overexpression.148 In line with these observations, an important product of lipid beta oxidation, beta-hydroxybutyrate, along with the level of carnitine, a critical modulator of fatty acid transport across the mitochondrial membrane, were decreased in both experimental groups.