Extracellular matrix abnormalities have been found in both human and animal models of
polycystic kidney disease (PKD). A new mouse PKD model has been produced through
insertion of a PGKneo cassette in an intron of the gene that encodes laminin α5 (Lama5), a
major tubular and glomerular basement membrane component that is important for
glomerulogenesis and ureteric bud branching. Lama5 neo represents a hypomorphic allele
as a result of aberrant splicing. Lama5 neo/neo mice exhibit PKD, proteinuria, and death …