Nucleoporins (NUPs) are an essential component of the nuclear-pore complex, which regulates nucleocytoplasmic transport of macromolecules. Pathogenic variants in NUP genes have been linked to several inherited human diseases, including a number with progressive neurological degeneration. NUP188 encodes a scaffold component of the nuclear pore complex (NPC) and has been implicated as a congenital heart disease gene through an ill-defined function at centrioles. NUP188 is specifically required for heart development. NUP188 also moonlights as a centrosome protein by binding directly to Cep152 and contributing to centriole duplication. Nup188 localizes to centrosomes, the major microtubule organizing centers in mammalian cells.