The molecular mechanisms maintaining glomerular filtration barrier are under intensive
study. This study describes a mutant Nphs1 mouse line generated by gene-trapping.
Nephrin, encoded by Nphs1, is a structural protein of interpodocyte filtration slits crucial for
formation of primary urine. Nephrin trap/trap mutants show characteristic features of
proteinuric disease and die soon after birth. Morphologically, fibrotic glomeruli with distorted
structures and cystic tubular lesions were observed, but no prominent changes in the …