Within the Elongator complex, ELP1/IKAP appears to act as a scaffoldprotein required for Elongator assembly and also serves to dock other proteins thatregulate the Elongator function, whereas ELP3 is the catalytic subunit and acetylateshistones H3 and H4 (Glatt and Muller, 2013).Consistent with these observations, IKAP protein is also observed in the nucleus, anddecreased IKAP expression in FD patient-derived fibroblasts leads to reduced ELP3levels, reduced Elongator binding, and decreased histone H3 acetylation in the codingregion of genes that are downregulated in FD cells. Here, ELP1 is linked to Fabry disease.