Mutations in the gene for integrin α3 (ITGA3), the main integrin linking podocyte foot processes to the glomerular basement membrane, cause junctional EB with interstitial lung disease and renal anomalies (ILNEB, OMIM#614748) including congenital nephrotic syndrome, focal segmental glomerulosclerosis, bilateral renal cysts, and a spectrum of congenital anomalies of the kidney and urinary tract (CAKUT) [13,21]. The gene discussed is ITGA3; the disease is familial nephrotic syndrome.