Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2Grc mutation
(1 available);
any
Ppp3r1 mutation
(30 available)
Tg(Pax3-cre)1Joe mutation
(0 available)
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renal/urinary system
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• abnormal pyeloureteral peristalsis resulting from kidney and ureter developmental defects
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• abnormal development of the renal pelvis due to impaired mesenchymal cell proliferation in the developing urinary tract, resulting in abnormal pyeloureteral peristalsis
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• first evident at 5 days of age and becoming more severe by 12 days of age
• in most cases the obstruction occurred at the level of the ureteropelvic junction
• associated with severe parenchymal atrophy, massive nephron loss, interstitial fibrosis, severe dilation of the tubular and pelvicaliceal space
• putatively due to abnormal the pyeloureteral peristalsis that resulted from the kidney and ureter developmental defects
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• severe parenchymal atrophy and massive nephron loss
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• abnormal development of the ureter due to impaired mesenchymal cell proliferation in the developing urinary tract, resulting in abnormal pyeloureteral peristalsis
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• in most cases the obstruction occurred at the level of the ureteropelvic junction
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• renal failure following progressive renal obstruction
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homeostasis/metabolism
cellular
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• decreased proliferation of mesenchymal cells along the urinary tract
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muscle
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• abnormal pyeloureteral peristalsis resulting from kidney and ureter developmental defects
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|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2.1Grc mutation
(0 available);
any
Ppp3r1 mutation
(30 available)
Ppp3r1tm2Grc mutation
(1 available);
any
Ppp3r1 mutation
(30 available)
Tg(Pax3-cre)1Joe mutation
(0 available)
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|
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renal/urinary system
|
|
• abnormal pyeloureteral peristalsis resulting from kidney and ureter developmental defects
|
|
|
• abnormal development of the renal pelvis due to impaired mesenchymal cell proliferation in the developing urinary tract
|
|
|
• first evident at 5 days of age and becoming more severe by 12 days of age
• in most cases the obstruction occurred at the level of the ureteropelvic junction
• associated with severe parenchymal atrophy, massive nephron loss, interstitial fibrosis, severe dilation of the tubular and pelvicaliceal space
• putatively due to abnormal the pyeloureteral peristalsis that resulted from the kidney and ureter developmental defects
|
|
|
• severe parenchymal atrophy and massive nephron loss
|
|
|
• abnormal development of the ureter due to impaired mesenchymal cell proliferation in the developing urinary tract
|
|
|
• in most cases the obstruction occurred at the level of the ureteropelvic junction
|
|
|
• renal failure following progressive renal obstruction
|
homeostasis/metabolism
cellular
|
|
• decreased proliferation of mesenchymal cells along the urinary tract
|
muscle
|
|
• abnormal pyeloureteral peristalsis resulting from kidney and ureter developmental defects
|
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2Grc mutation
(1 available);
any
Ppp3r1 mutation
(30 available)
Tg(GATA5-cre)1Krc mutation
(0 available)
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cardiovascular system
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N |
• mice are grossly normal and display no defects in endothelial patterning at E12.5; mice live to adulthood and are indistinguishable from wild-type littermates
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2.1Grc mutation
(0 available);
any
Ppp3r1 mutation
(30 available)
Ppp3r1tm2Grc mutation
(1 available);
any
Ppp3r1 mutation
(30 available)
Tg(Lck-cre)1Cwi mutation
(3 available)
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hematopoietic system
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• double positive (DP) thymocytes cannot progress to the single positive (SP)
• DP thymocytes show defects in induction of TCRb, CD69, and CD5 which are markers of positive selection
• negative selection was unaffected
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• decreased numbers of T lymphocytes compared to controls
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immune system
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• double positive (DP) thymocytes cannot progress to the single positive (SP)
• DP thymocytes show defects in induction of TCRb, CD69, and CD5 which are markers of positive selection
• negative selection was unaffected
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• decreased numbers of T lymphocytes compared to controls
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• decreased numbers of T lymphocytes compared to controls
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homeostasis/metabolism
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|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2Grc mutation
(1 available);
any
Ppp3r1 mutation
(30 available)
Tg(Lck-cre)1Cwi mutation
(3 available)
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hematopoietic system
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• general decrease in cellularity is observed
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• specific reduction in CD4+CD8- and CD4-CD8+ single positive (SP) thymocytes is found
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immune system
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• general decrease in cellularity is observed
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• specific reduction in CD4+CD8- and CD4-CD8+ single positive (SP) thymocytes is found
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endocrine/exocrine glands
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• general decrease in cellularity is observed
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• specific reduction in CD4+CD8- and CD4-CD8+ single positive (SP) thymocytes is found
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2Grc mutation
(1 available);
any
Ppp3r1 mutation
(30 available)
Tg(Tagln-cre)1Her mutation
(2 available)
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cardiovascular system
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N |
• mice are grossly normal and display normal coronary endothelial patterning at E12.5; mice live to adulthood and are indistinguishable from wild-type littermates
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2Grc mutation
(1 available);
any
Ppp3r1 mutation
(30 available)
Tg(Tek-cre)1Ywa mutation
(6 available)
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mortality/aging
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• most embryos die at E13 due to heart valve defects
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cardiovascular system
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N |
• peripheral vasculature is not observably different from wild-type at E10.5 and 11.5; cranial, intersomitic and dorsal vessels appear normal at E10.5 and 11.5
• at E12.5, no significant differences in endothelial cell proliferation or apoptosis are detected
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• coronary endothelial cells are fused and limited to the atrioventricular junction; PECAM1-positive endothelial cells are limited to basal portion of ventricles with no cells reaching the apical region of the heart
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• hearts at E12.5 show limited endothelial branching compared to extensive network of endothelial tubes in wild-type
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Analysis Tools