Search Criteria: Research Area is "Mouse/Human Gene Homologs: Alport syndrome"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 006183 | B6.Cg-Col4a5tm1Yseg/J | Repository- Live |
| Heterozygous females and hemizygous males for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Immunofluorescence analysis of kidney tissue from mutant male mice does not detect gene product (protein), but in kidney from mutant female mice a mosaic pattern of gene product (protein) is detected (due to X-inactivation). RNase protection assay analysis of kidney tissue from mutant male mice detects a significantly lower level than wildtype. Hemizygous male and heterozygous female mice develop progressive kidney disease. By 7 weeks of age, 97% of mutant male mice and 78% of heterozygous female mice exhibit proteinuria. Histological analysis of kidneys from mutant male mice at 4 weeks of age reveals thickened capillary walls and mesangial hypercellularity. The pathology progresses to interstitial inflammation, glomerular abnormalities, and focal sclerosis. Electron microscopic examination of glomerular basement memb
..... For more information please see the full descriiption on the strain data sheet | ||
| 002908 | 129-Col4a3tm1Dec/J | Repository-Cryopreserved |
| Mice homozygous for the Col4a3tm1Dec targeted mutation develop glomerulonephritis and die at about 8.5 weeks of age. Survival time of homozygous mutant mice is extended to about 14 weeks of age in mice maintained on a mixed genetic background. | ||
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