Phenotypes for Lama5 MGI:3823250 MGI Mouse

premature death ( J:128225 )

• mice die by day 28

• cystic epithelia exhibit increased apoptosis

polycystic kidney ( J:128225 )

• by P14, mice develop numerous renal cysts

• in some cysts, epithelial cells loss their unilayered orientation and pile up on one another

• most cysts originate from the proximal tubules while others involve cells from the ascending limb of Henle and the collecting duct

• however, cilia are present in cyst epithelial cells and cysts do not form in the liver or pancreas

• cysts epithelial cells lack tubular cell features such as a dense brush border, and the underlying basement membrane is initially uniform, thickened and exhibits overlays of fibrillar collagen deposits at advanced stages

• cystic epithelia exhibit increased cell proliferation and apoptosis

enlarged kidney ( J:128225 )

• in some mice

increased urine protein level ( J:128225 )

• as early as P2 to P4

• protein to creatine levels in the urine are increased compared to in wild-type mice

abnormal renal glomerulus basement membrane morphology ( J:128225 )

• mice exhibit glomerular basement membrane defects such as thickening of the lamina rara interna and splitting

renal interstitial fibrosis ( J:128225 )

• at later ages

dilated renal tubule ( J:128225 )

• as early as P1 to P2

kidney failure ( J:128225 )

• by P21, mice exhibit progressive

increased blood urea nitrogen level ( J:128225 )