Unknown,Transcriptomics,Genomics,Proteomics

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Transcription profiling of mouse renal epithelial cells from inv mutant animals

ABSTRACT: 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). Experiment Overall Design: 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

ORGANISM(S): Mus musculus

SUBMITTER: Noriyuki Sugiyama

PROVIDER: E-GEOD-4462 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

Sustained cell proliferation of renal epithelial cells in mice with inv mutation.

Sugiyama Noriyuki N Yokoyama Takahiko T

Genes to cells : devoted to molecular & cellular mechanisms 20061001 10

A tubule system is an important component of the nephron, which is the structural and functional unit of the kidney. Expansion of renal tubules results in renal cysts. Hereditary forms of renal cystic diseases suggest that tubular size is determined genetically. The inv was discovered as a mutant with renal cysts and situs inversus. Inv/inv, inv deltaC::GFP (inv deltaC) mouse was created by the introduction of the inv gene lacking the C-terminus (inv deltaC) into inv/inv mice. The mouse develops ...[more]

PMID: 16999740

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Project description:Polycystic Kidney Disease is characterized by the formation of large fluid-filled cysts that eventually destroy the renal parenchyma leading to end-stage renal failure. Although remarkable progress has been made in understanding the pathologic mechanism of the disease, the precise orchestration of the early events leading to cyst formation is still unclear. Abnormal cellular proliferation was traditionally considered to be one of the primary irregularities leading to cyst initiation and growth. Consequently, many therapeutic interventions have focused on targeting this abnormal proliferation, and some have even progressed to clinical trials. However, the role of proliferation in cyst development was primarily examined at stages where cysts are already visible in the kidneys and therefore at later stages of disease development. In this study we focused on the cystic phenotype since birth in an attempt to clarify the temporal contribution of cellular proliferation in cyst development. Using a PKD2 transgenic rat model (PKD2 (1-703)) of different ages (0-60 days after birth) we performed gene expression profiling and phenotype analysis by measuring various kidney parameters. Phenotype analysis demonstrated that renal cysts appear immediately after birth in the PKD2 transgenic rat model (PKD2 (1-703)). On the other hand, abnormal proliferation occurs at later stages of the disease as identified by gene expression profiling. Interestingly, other pathways appear to be deregulated at early stages of the disease in this PKD model. Our data suggest that cystogenesis precedes deregulation of proliferation-related pathways, suggesting that proliferation abnormalities may contribute in cyst growth rather than cyst formation. In this study we focused on the cystic phenotype since birth in an attempt to clarify the temporal contribution of cellular proliferation in cyst development. Using a PKD2 transgenic rat model (PKD2 (1-703)) of different ages (0,6 and 24 days after birth) we performed gene expression profiling of kidney cells.

Dataset Information

Transcription profiling of mouse renal epithelial cells from inv mutant animals

Publications

Sustained cell proliferation of renal epithelial cells in mice with inv mutation.

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