NUP205 (nucleoporin 205) is a critical structural component of the nuclear pore complex (NPC) that serves multiple essential cellular functions. As part of the inner ring of the NPC, NUP205 plays a fundamental role in nuclear pore assembly and maintenance, with two copies present in each cytoplasmic ring subunit 1. The protein anchors various nucleoporins to the NPC and mediates contacts between neighboring cytoplasmic ring subunits through interactions with NUP93 1. Beyond its structural role, NUP205 is essential for nucleocytoplasmic transport, specifically regulating nuclear import of transcriptional effectors YAP and TAZ in the Hippo pathway 2. NUP205 also functions in cilia biology, where its depletion results in cilia loss and abnormal cardiac morphology, contributing to proper left-right patterning during embryonic development 3. Clinically, pathogenic variants in NUP205 cause autosomal recessive steroid-resistant nephrotic syndrome (SRNS) and focal segmental glomerulosclerosis, typically presenting around age 2 years with progression to kidney failure by age 7 years 4. Additionally, NUP205 variants are associated with congenital heart disease and situs defects, linking the protein to ciliopathy pathogenesis 5.