Mutations in the human Elp1 homolog IKAP (also known as ELP1) causes familial dysautonomia, a rare neurodegenerative disease that is associated with growth abnormalities and degradation of sensory functions, and patients suffering from this disease show reduced levels of the wobble uridine tRNA modification (Dalwadi and Yip, 2018). Here, ELP1 is linked to neurodegenerative disease.