NUP93 is a nucleoporin that functions as a structural scaffold component of the nuclear pore complex (NPC), playing critical roles in NPC assembly and maintenance 1. Mechanistically, NUP93 contains an N-terminal assembly sensor motif that anchors the cytoplasmic filament nucleoporin complex to the NPC's cytoplasmic face 2, and bridges Y-complex components with other nucleoporins like NUP205 to mediate contacts between neighboring NPC subunits 3. Beyond canonical NPC functions, NUP93 regulates nuclear organization by tethering the HOXA gene locus to the nuclear periphery during differentiation, modulating spatiotemporal gene expression dynamics 4. NUP93 also regulates podocyte migration and proliferation through SMAD4 signaling during renal development. Clinically, NUP93 mutations cause steroid-resistant nephrotic syndrome (SRNS), a monogenic form with median disease onset at age 3 years 5. Pathogenic variants result in focal segmental glomerulosclerosis in ~89% of patients, progressing to kidney failure at median age 3.7 years 5. NUP93 is expressed throughout human kidney nuclei in both glomerular and tubulointerstitial cells, with patient mutations significantly reducing nuclear NUP93 levels 6. Beyond renal disease, NUP93 dysfunction contributes to amyotrophic lateral sclerosis through selective NPC component loss and oxidative damage-driven carbonylation affecting nucleocytoplasmic transport 7. Management includes renin-angiotensin-aldosterone system inhibitors, immunosuppressant discontinuation following genetic diagnosis, and kidney transplantation at end-stage renal disease.