Nephronophthisis (NPHP) is a group of progressive renal disorders characterized by a variable number of cysts associated with cortical tubular atrophy. Recently, mutations in INV have been identified to be responsible for NPHP2. The inv/inv,inv-deltaC::GFP mouse that was created by introduction of inv gene lacking the C-terminus (inv-deltaC) into inv/inv mice develops multiple renal cysts without situs abnormality. Renal cyst progression was dependent on expression level of inv-deltaC transgene, suggesting that inv protein functions in a dose-dependent manner. Cell proliferation and apoptosis were increased in the inv-deltaC cystic kidneys. We searched up- and down-regulated genes during cyst development and progression using cDNA microarray.
See appended table for comparison analysis of inv/inv,inv-deltaC::GFP vs +/+,inv-deltaC::GFP (GSM100334 vs GSM100335). Keywords: apoptosis, cell proliferation, gene expression, inv, inversin, polycystic kidney disease
Comparison of gene expression in each two kidneys in 4-week-old inv-deltaC and control mice was carried out by the KURABO Industries, Biomedical Department