The importance of this structural region for Abl’s function is highlighted by several mutations in the αI’-helix, including E528K at its C-terminal end associated with the recently described ABL1 malformation syndrome leading to congenital heart disease, skeletal malformations, characteristic facies, and hearing impairment (Wang et al., 2017; Blakes et al., 2021). The gene discussed is ABL1; the disease is congenital heart disease.