Nuclear pore complex protein Nup133 (NUP133)

Nuclear pore complex protein Nup133 (NUP133) is a structural nucleoporin essential for the assembly and function of the nuclear pore complex (NPC) in eukaryotic cells[1][2][3][7][8]. It participates in forming the Y-shaped nucleoporin subcomplex (Nup107–160 complex), localized peripherally at the NPC and is tightly associated with Nup107[2][3]. Nup133 plays a key role in nucleocytoplasmic transport by contributing to the selective passage of macromolecules across the nuclear envelope[1][2][3][7]. Its N-terminal domain forms a seven-bladed β-propeller, supporting multiple protein-protein interactions important for structural integrity and NPC biogenesis[3]. Loss of Nup133 disrupts NPC assembly, resulting in defects in gene expression regulation, nuclear architecture, and increased susceptibility to nuclear envelope rupture under stress, especially in neuronal and renal tissues[5][7][8]. Mutations in NUP133 are linked to specific genetic diseases, including Galloway-Mowat syndrome 8 and nephrotic syndrome type 18, demonstrating its critical roles in development and organ function[7][8]. There are no known drugs directly targeting Nup133, and it is not considered a canonical therapeutic target, but its dysfunction is of clinical and biological significance.

Other names

Nucleoporin Nup133133 kDa nucleoporinGAMOS8NPHS18hNUP133FLJ10814nucleoporin 133kDnucleoporin 133kDa