AAAS and achalasia-alacrima syndrome: ALADIN is regulated by phosphorylation and is frequently targeted for mutation in the triple A syndrome (AAAS).27–29 All three potential phosphorylation sites (Ser186, Thr295, and Ser300) in ALADIN24,25 are located on surface loops on the top side of the β-propeller (Supplementary information, Fig. S19a); these residues are likely involved in protein recruitment or interaction that might be sabotaged by mutations.