NUP133 (nucleoporin 133) is a structural component of the nuclear pore complex (NPC) that mediates nucleocytoplasmic transport and regulates gene expression 1. NUP133 functions as part of the heptameric Y-complex scaffold, where it interacts tightly with NUP107 in a tail-to-tail fashion to position NUP133 at the NPC periphery 2. The protein contains a conserved amphipathic lipid packing sensor motif involved in membrane anchoring 3. NUP133 plays critical roles in nuclear pore assembly, transcriptome regulation, and cytoskeleton maintenance in podocytes 4. Pathogenic NUP133 variants cause autosomal recessive steroid-resistant nephrotic syndrome (SRNS) with median disease onset at 9 years, progressing to kidney failure at median age 13 years 1. NUP133 mutations also cause Galloway-Mowat syndrome (GAMOS), characterized by microcephaly, brain malformations, and early-onset nephrotic syndrome 5. Loss of NUP133 function impairs protein interactions within the Y-complex and disrupts nuclear pore assembly, leading to altered podocyte transcriptomes and impaired cellular protrusion generation 4. Kidney biopsies reveal focal segmental glomerulosclerosis in affected patients 1. Genetic diagnosis via targeted sequencing and management with renin-angiotensin-aldosterone system inhibitors are recommended, with kidney transplantation preferred for end-stage renal disease 1.