Analysis ToolsMouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| progressive pseudorheumatoid arthropathy of childhood | DOID:0090004 |
OMIM:208230 |
J:201718 | |
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Allele Symbol Allele Name Allele ID |
Ccn6tm1(cre)Mawa targeted mutation 1, Matthew L Warman MGI:5302829 |
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| Summary |
2 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| progressive pseudorheumatoid arthropathy of childhood | DOID:0090004 |
OMIM:208230 |
J:201718 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
 |
• males show a ~5-fold reduction in cauda epididymal sperm number relative to control males
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• males show a ~3-fold increase in morphologically abnormal cauda epididymidal sperm relative to control males
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• 63% of sperm show midpiece curling
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• 29% of sperm have bent tails, with flagella bent or folded at the annulus
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• 2% of sperm have multiple tails
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• 6% of sperm show head malformations
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• males show a 3-fold increase in spermatocyte apoptosis, as determined by co-staining with cleaved cPARP1 (a marker for apoptosis) and SYCP3 (a spermatocyte marker)
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• adult testes are ~30% smaller than control testes
• however, testis histology shows complete spermatogenesis with supporting cells and multiple generations of germ cells
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• adult testis weight is significantly lower than in control males
• however, adult body weight is normal
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• when adult males are bred with wild-type females for 4 months, average litter size is significantly lower than in control males
• however, total number of litters is normal
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• sperm lysates exhibit significantly reduced PPP1CC (protein phosphatase 1 catalytic subunit gamma, aka PP1) activity relative to lysates from control sperm
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• specific sperm motility parameters, including % progressive motility, path velocity (VAP), curvilinear velocity (VCL) and relative motility index (RMI = % motility x straight line velocity (VSL)/100), are all significantly increased relative to controls
• however, % total motility and other parameters including lateral amplitude (ALH), beat frequency (BCF), straightness (STR), and linearity (LIN) are not significantly altered
|
|
|
• sperm progressive motility is increased by 25% relative to that in control males
|
|
|
• males show a ~5-fold reduction in cauda epididymal sperm number relative to control males
|
|
|
• males show a ~3-fold increase in morphologically abnormal cauda epididymidal sperm relative to control males
|
|
|
• 63% of sperm show midpiece curling
|
|
|
• 29% of sperm have bent tails, with flagella bent or folded at the annulus
|
|
|
• 2% of sperm have multiple tails
|
|
|
• 6% of sperm show head malformations
|
|
|
• males show a 3-fold increase in spermatocyte apoptosis, as determined by co-staining with cleaved cPARP1 (a marker for apoptosis) and SYCP3 (a spermatocyte marker)
|
|
|
• specific sperm motility parameters, including % progressive motility, path velocity (VAP), curvilinear velocity (VCL) and relative motility index (RMI = % motility x straight line velocity (VSL)/100), are all significantly increased relative to controls
• however, % total motility and other parameters including lateral amplitude (ALH), beat frequency (BCF), straightness (STR), and linearity (LIN) are not significantly altered
|
|
|
• sperm progressive motility is increased by 25% relative to that in control males
|
|
|
• adult testes are ~30% smaller than control testes
• however, testis histology shows complete spermatogenesis with supporting cells and multiple generations of germ cells
|
|
|
• adult testis weight is significantly lower than in control males
• however, adult body weight is normal
|
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 04/23/2024 MGI 6.23 |
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