Analysis Tools|
Allele Symbol Allele Name Allele ID |
Slc25a13tm1Lct targeted mutation 1, Lap-Chee Tsui MGI:3028639 |
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| Summary |
4 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• under fasting and fed conditions
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• under fed conditions
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• following 16 hours of fasting
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• following treatment with salt or NH4Cl, hepatic ammonia, glutamic acid, and glutamine levels are elevated compared to in similarly treated wild-type mice
• following treatment with NH4Cl, aspartic acid levels are higher than in similarly treated wild-type mice
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• when the liver is perfused with lactate, glucose production is reduced compared to in similarly treated wild-type mice
• however, glucose production following hepatic perfusion with pyruvate is normal
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• following treatment with NH4Cl, mice exhibit reduced urea production compared with similarly treated wild-type mice
• however, urea production can be partially rescued by treatment with asparagine
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• following treatment with NH4Cl, mice exhibit reduced urea production compared with similarly treated wild-type mice
• however, urea production can be partially rescued by treatment with asparagine
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| NOT | citrullinemia | DOID:9273 | J:87578 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• following treatment with NH4Cl, glutamic acid levels are higher than in similarly treated wild-type mice
• following treatment with salt or NH4Cl, glutamine levels are higher than in similarly treated wild-type mice
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• when the liver is perfused with lactate, glucose production is reduced compared to in wild-type mice although not as much as in homozygous mice
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• 30% fewer than expected mice are found between 20 and 40 days
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• hepatic aspartic acid levels are higher than in wild-type mice
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• citrulline and threonine/serine ratios are higher than in wild-type mice
• following oral sucrose administration, the plasma alanine concentration and branched amino acid/aromatic amino acid ratio are decreased compared to in wild-type mice
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• under fed conditions and after oral sucrose administration
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• plasma urea concentrations are higher than in wild-type mice
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• compared to in wild-type mice and more severely than in Slc25a13tm1Lct homozygotes under fasting and fed conditions
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• compared to wild-type mice and more severely than in Gpd2tm1Tka homozygotes
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• following 16 hours of fasting
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• by 36 days in males and 29 days in females
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| citrullinemia | DOID:9273 | J:124766 | ||
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 09/30/2025 MGI 6.24 |
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