About   Help   FAQ
Phenotypes Associated with This Genotype
Genotype
MGI:7442509
Allelic
Composition
Srgap1tm1a(KOMP)Wtsi/Srgap1tm1a(KOMP)Wtsi
Six2tm1(tTA,tetO-EGFP/cre)Amc/Six2+
Genetic
Background
involves: 129 * C57BL/6J * C57BL/6N
Cell Lines EPD0153_3_C10
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Six2tm1(tTA,tetO-EGFP/cre)Amc mutation (0 available); any Six2 mutation (16 available)
Srgap1tm1a(KOMP)Wtsi mutation (1 available); any Srgap1 mutation (74 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• increased mesangial cell proliferation at 12 weeks of age
• podocyte detachment at 12 weeks of age
• progressive albuminuria, starting at 6 weeks after birth
• however, no increase in proteinuria is detected at birth
• TEM analysis showed pronounced widening and misconfiguration (effacement) of podocyte foot processes at 8 and 12 weeks of age
• 3D SIM microscopy showed reduced filtration slit density (FSD), indicating aberrant foot process architecture at 8 weeks of age
• SEM revealed marked simplification, reduced branching, and misconfiguration of podocyte foot processes
• marked rarefaction and loss of slit diaphragms at 12 weeks of age
• progressive podocyte loss
• mesangial sclerosis at 12 weeks of age
• mice develop a nephrotic syndrome with a late-onset focal segmental glomerulosclerosis (FSGS)-like phenotype in adulthood
• a significantly increased glomerulosclerosis score is noted at 8 and 12 weeks of age
• tubular dilation at 12 weeks of age
• formation of proteinaceous casts at 12 weeks of age
• progressive loss of filtration-barrier function, starting at 6 weeks after birth

cellular
• increased mesangial cell proliferation at 12 weeks of age
• podocyte detachment at 12 weeks of age

homeostasis/metabolism
• progressive albuminuria, starting at 6 weeks after birth
• however, no increase in proteinuria is detected at birth

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
focal segmental glomerulosclerosis DOID:1312 OMIM:PS603278
J:333727


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)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
12/10/2024
MGI 6.24
The Jackson Laboratory