All Phenotypes Arl13b<tm1c(EUCOMM)Wtsi> MGI Mouse

premature death ( J:240795 )

• mice die at around P60

absent kidney epithelial cell primary cilium ( J:240795 )

• at P14, most DBA-positive collecting duct epithelial cells are devoid of cilia, indicating a cilia biogenesis defect

increased kidney cell proliferation ( J:240795 )

• at P14, kidneys exhibit ~14% of BrdU-positive nuclei in DBA-positive cyst-lining cells versus ~2.8% in control kidneys, suggesting a significant increase in cell proliferation

kidney cyst ( J:240795 )

• mice show rapid kidney cyst formation, with minimal cysts at P7, significant cysts in both the medulla and cortex region at P14, and severely cystic kidneys at P17

• cyst formation is confined to the distal nephron

• at P14, cysts mainly form in the collecting duct; by P28, cysts are detected in the medullary thick ascending limb and the distal convoluted tubule

kidney cortex cyst ( J:240795 )

• significant cysts in the cortex region at P14

kidney medulla cyst ( J:240795 )

• significant cysts in the medulla region at P14

polycystic kidney ( J:240795 )

• mice exhibit rapid progression of polycystic kidney disease (PKD) with severely cystic kidneys at P17

• treatment with valproic acid (VPA, a histone deacetylase inhibitor) can partially suppress PKD progression

enlarged kidney ( J:240795 )

• starting at P14, kidney size is obviously enlarged; by P17, kidney size is significantly increased

• mice treated with valproic acid (VPA, a histone deacetylase inhibitor) from P10 to P25 show significantly reduced kidney size relative to vehicle-treated controls

increased kidney weight ( J:240795 )

• at P14, kidney-to-body weight ratio is significantly increased

• mice treated with VPA from P10 to P25 show a significantly reduced kidney-to-body weight ratio relative to vehicle-treated controls

dilated kidney collecting duct ( J:240795 )

• at P7, some DBA-positive regions marking the collecting ducts are already dilated

• in dilated regions, cilia are largely absent, whereas cilia are still present in non-dilated DBA-positive regions

renal fibrosis ( J:240795 )

• at P28, but not at P21, kidneys exhibit increased trichrome staining indicating collagen deposition

• mice treated with VPA from P10 to P25 show a significantly reduced collagen deposition relative to vehicle-treated controls

renal interstitial fibrosis ( J:240795 )

• at P28, kidney interstitial cells show a dramatic increase in smooth muscle actin expression relative to controls

• VPA treatment from P10 to P25 significantly reduces smooth muscle actin expression in the interstitium

kidney failure ( J:240795 )

• mice develop rapidly progressive renal failure

increased blood urea nitrogen level ( J:240795 )

• BUN level is significantly increased at P14

• mice treated with VPA from P10 to P25 show a significantly reduced BUN level, indicating improved kidney function