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Phenotypes Associated with This Genotype
Genotype
MGI:6357739
Allelic
Composition
Atp6ap2tm1.1Aich/Atp6ap2tm1.1Aich
Tg(Six2-EGFP/cre)1Amc/0
Genetic
Background
involves: C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp6ap2tm1.1Aich mutation (0 available); any Atp6ap2 mutation (10 available)
Tg(Six2-EGFP/cre)1Amc mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die within 48 hours of birth

renal/urinary system
• kidneys have many cysts in the cortex and outer medulla at P0
• thin kidney cortex at P0
• kidneys have many cysts in the cortex and outer medulla at P0
• decreased nephrogenesis, with premature depletion of the progenitor cell population within the cap mesenchyme
• the cap mesenchyme shows fewer proliferating cells, however apoptosis is no different from controls
• marker analysis shows reduced inductive and differentiation response within the metanephric mesenchyme
• kidneys lack a well-defined nephrogenic zone at P0
• kidneys are reduced in size at E13.5 and in newborns
• kidney weight is lower in newborns
• nephron structure volume compared to total kidney volume is reduced at E12.5
• developing glomeruli volume and average size are reduced at E12.5
• nascent nephrons reach the renal vesicle stage but do not develop beyond
• kidneys show less developed epithelial structures at E13.5, with embryos having few vesicles and comma-shaped nephron structures
• all stages of nephron development are reduced at P0 and marker analysis shows that nephron induction and specification of proximal nephron segments are impaired at birth
• the number of S-shaped bodies is reduced at E12.5
• however, renal vesicle number is not different at E12.5
• the number of comma-shaped bodies is reduced at E12.5
• decrease in the number of nephrons at P0
• ureteric bud branching is reduced at E13.5

growth/size/body
• kidneys have many cysts in the cortex and outer medulla at P0
• kidneys have many cysts in the cortex and outer medulla at P0


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
03/18/2025
MGI 6.24
The Jackson Laboratory