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Nuclear pore complex protein Nup205 (NUP205)
- Target
- NUP205
- Molecular classification
- Nucleoporin (nuclear pore protein), Nuclear pore complex component, Inner ring subunit
01
Overview
Nuclear pore complex protein Nup205 is an inner ring component of the nuclear pore complex essential for the assembly, maintenance, and function of the NPC. It facilitates active transport of proteins, RNA, and ribonucleoprotein particles between the nucleus and cytoplasm. NUP205 is specifically required for the nuclear import and transcriptional activity of Yes-associated protein (YAP) and transcriptional co-activator with PDZ-binding motif (TAZ), linking it to the regulation of cell proliferation and apoptosis in podocytes. Mutations in NUP205 are implicated in steroid-resistant nephrotic syndrome, focal segmental glomerulosclerosis, and certain ciliopathies—including congenital heart disease—arising from defective cilia formation[1][2][3][4].
- Other names
- C7orf14KIAA0225205 kDa nucleoporinNucleoporin Nup205NPHS13
02
Mechanism of action
Not established for pharmaceuticals; however, NUP205 modulates the nuclear import of transcriptional regulators (YAP/TAZ)[1].
03
Biological functions
Nucleocytoplasmic transport (proteins, RNAs, ribonucleoproteins)Nuclear pore complex assembly and maintenanceRegulation of nuclear import of transcription factors (YAP and TAZ)Role in cilia biology and organ patterningCell proliferationAnti-apoptotic protection in podocytes
04
Disease associations
Steroid-resistant nephrotic syndrome (SRNS)Focal segmental glomerulosclerosis (FSGS)Hereditary nephrotic syndrome, Type 13Ciliopathy-related congenital heart disease
05
Safety considerations
genetic disruption leading to podocyte injuryincreased cell deathcompromised cell proliferationdysregulation of cilia biogenesiscongenital organ defects
06
Biomarkers
Mutations in NUP205 act as genetic biomarkers for steroid-resistant nephrotic syndrome and FSGS[1][2][4].
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