NUP85 is an essential component of the nuclear pore complex (NPC) that plays critical roles in cellular transport and development 1. As part of the Nup107-160 subcomplex, NUP85 is required for NPC assembly and maintenance, RNA export, and tethering other nucleoporins to the nucleus 1. The protein also functions in spindle assembly during mitosis and is involved in nephrogenesis 1. Pathogenic variants in NUP85 cause autosomal recessive steroid-resistant nephrotic syndrome (SRNS), with patients typically presenting focal segmental glomerulosclerosis at kidney biopsy 12. The phenotypic spectrum has expanded beyond nephropathy to include primary autosomal recessive microcephaly and Seckel syndrome spectrum disorders, characterized by intellectual disability, growth retardation, and neurological features 345. NUP85-associated SRNS typically manifests around age 7 years and progresses to kidney failure by median age 8.5 years, with most patients showing resistance to immunosuppressive therapy 1. The protein appears critical for brain development, as mutations cause severe neurodevelopmental impairment including microcephaly, seizures, and corpus callosum abnormalities 5. Recent studies also implicate NUP85 in metabolic dysfunction-associated steatotic liver disease, suggesting broader physiological roles 6.