NUP188 is a scaffold nucleoporin component of the nuclear pore complex (NPC) with critical roles in both canonical and non-canonical cellular functions. Primary function: NUP188 is essential for nuclear import of proteins, with bi-allelic loss-of-function causing decreased nuclear protein import in patient fibroblasts 1. As a structural constituent of the NPC inner ring, NUP188 forms question mark-shaped keystones within hetero-octameric complexes that establish NPC integrity while allowing conformational flexibility of the central transport channel 2. Beyond NPC roles, NUP188 localizes to centrosomes where differential protein turnover regulates its steady-state levels and functions in centriole duplication upstream of Sas6 loading 3. Non-classical function: NUP188 knockdown results in cilia loss, and genetic variants have been identified in patients with congenital heart disease and heterotaxy 4. Disease relevance: Bi-allelic NUP188 truncating variants cause Sandestig-Stefanova syndrome, characterized by prenatal ventriculomegaly, white-matter abnormalities, congenital cataracts, cardiac defects, and central hypoventilation 1. NUP188 is dysregulated across multiple cancer types, with elevated expression associated with poor prognosis in gastric and muscle-invasive bladder cancers and linked to metabolic reprogramming 56. Additionally, NUP188 assists Nup93 in repressing HOXA gene expression through chr9 organization 7.