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Phenotypes Associated with This Genotype
Genotype
MGI:6402523
Allelic
Composition
Cmastm1.1Bwei/Cmastm1.1Bwei
Tg(NPHS2-cre)295Lbh/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cmastm1.1Bwei mutation (0 available); any Cmas mutation (28 available)
Tg(NPHS2-cre)295Lbh mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• dilatation and narrowing at P42

growth/size/body
• starting at P28
• starting at P28

homeostasis/metabolism
• progressive, starting around P28

immune system
• eosinophilic material in some proximal and distal tubules at P28

mortality/aging
• between postnatal weeks 6 and 8

renal/urinary system
• dilatation and narrowing at P42
• at P42, mice exhibit mesangial cell expansion and activation in the smooth muscle cell of blood vessels, the center of the glomerular tufts (P57), and in Bowmans capsule (P42 and P57) unlike control mice
• progressive, starting around P28
• eosinophilic material in some proximal and distal tubules at P28
• resulting in podocyte fusion, complete loss of slit diaphragm and microvillous transformation at P42
• with reduction in mature slit diaphragm at P28
• focal segmental glomerulosclerosis (FSGS) with some glomeruli severely affected while others have no signs of pathology
• however, mice do not exhibit endothelial fenestration or altered glomeruli basement membrane morphology
• of glomerular tufts to Bowmans capsule with dilation of both at P42
• increased collagen deposition at P42
• reduced glomerular filtration rate at P28

cellular
• at P42, mice exhibit mesangial cell expansion and activation in the smooth muscle cell of blood vessels, the center of the glomerular tufts (P57), and in Bowmans capsule (P42 and P57) unlike control mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
focal segmental glomerulosclerosis DOID:1312 OMIM:PS603278
J:286022


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
03/19/2024
MGI 6.23
The Jackson Laboratory