Laminin β2 is a component of laminin-521, which is an important constituent of the
glomerular basement membrane (GBM). Null mutations in laminin β2 (LAMB2) cause
Pierson syndrome, a severe congenital nephrotic syndrome with ocular and neurologic
defects. In contrast, patients with LAMB2 missense mutations, such as R246Q, can have less
severe extrarenal defects but still exhibit congenital nephrotic syndrome. To investigate how
such missense mutations in LAMB2 cause proteinuria, we generated three transgenic lines …