Additionally, we identify a lipidomic pattern associated with the ALS‐FTLD‐TDP43 proteinopathy spectrum, notably characterised by down‐regulation of ether lipids and acylcarnitine among other lipid species, as well as alterations in the lipidome of each phenotype of TDP43 proteinopathy, which reveals commonalities and disease‐dependent differences in lipid composition. Here, TARDBP is linked to amyotrophic lateral sclerosis.