NUP Nephropathy: When Defective Pores Cause Leaky Glomeruli Beata S. Lipska-Ziętkiewicz, Franz Schaefer  American Journal of Kidney Diseases  Volume 73, Issue 6, Pages 890-892 (June 2019) DOI: 10.1053/j.ajkd.2019.01.015 Copyright © 2019 National Kidney Foundation, Inc. Terms and Conditions

Figure 1 Nuclear pore complexes (NPCs). (A) Schematic of NPCs, which are large channels across the nuclear envelope through which nucleocytoplasmic transport occurs, distributed on the nucleus (left image); in reality, there are more than 500 nuclear pores in each nucleus. NPCs are composed of several subcomplexes (each denoted here with a distinct color; right image): cytoplasmic filaments, cytoplasmic ring, central framework (inner ring), nuclear ring, and nuclear basket. (B) Architecture of the NPC. NPCs consist of more than 30 nucleoporin (NUP) proteins. Circles denote individual NUPs present in each substructure. Shaded circles correspond to NUPs involved in steroid-resistant nephrotic syndrome: NUP93, NUP205 (in inner ring) and NUP85, NUP107, NUP133, and NUP160 (forming the Y complex, a structural element of the outer ring with both cytosolic and nuclear elements). Graphics in panel A created with BioRender. American Journal of Kidney Diseases 2019 73, 890-892DOI: (10.1053/j.ajkd.2019.01.015) Copyright © 2019 National Kidney Foundation, Inc. Terms and Conditions