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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Slc25a13tm1Lct
targeted mutation 1, Lap-Chee Tsui
MGI:3028639
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
 		Slc25a13tm1Lct/Slc25a13tm1Lct B6.129-Slc25a13tm1Lct MGI:3852221
hm2
 		Slc25a13tm1Lct/Slc25a13tm1Lct either: 129/Sv-Slc25a13tm1Lct or (involves: 129S1/Sv * 129X1/SvJ * CD-1) MGI:3852334
ht3
 		Slc25a13tm1Lct/Slc25a13+ either: 129/Sv-Slc25a13tm1Lct or (involves: 129S1/Sv * 129X1/SvJ * CD-1) MGI:3852335
cx4
 		Gpd2tm1Tka/Gpd2tm1Tka
Slc25a13tm1Lct/Slc25a13tm1Lct 		B6.Cg-Gpd2tm1Tka Slc25a13tm1Lct MGI:3852220


Genotype
MGI:3852221
hm1
Allelic
Composition		 Slc25a13tm1Lct/Slc25a13tm1Lct
Genetic
Background		 B6.129-Slc25a13tm1Lct
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Slc25a13tm1Lct mutation (3 available); any Slc25a13 mutation (178 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
homeostasis/metabolism
hypoglycemia ( J:124766 )
• under fasting and fed conditions
decreased circulating insulin level ( J:124766 )
• under fed conditions

liver/biliary system
hepatic steatosis ( J:124766 )
• following 16 hours of fasting




Genotype
MGI:3852334
hm2
Allelic
Composition		 Slc25a13tm1Lct/Slc25a13tm1Lct
Genetic
Background		 either: 129/Sv-Slc25a13tm1Lct or (involves: 129S1/Sv * 129X1/SvJ * CD-1)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Slc25a13tm1Lct mutation (3 available); any Slc25a13 mutation (178 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
homeostasis/metabolism
abnormal amino acid level ( J:87578 )
• 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
abnormal gluconeogenesis ( J:87578 )
• 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
abnormal urine homeostasis ( J:87578 )
• 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

renal/urinary system
abnormal urine homeostasis ( J:87578 )
• 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

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
NOT citrullinemia DOID:9273 J:87578




Genotype
MGI:3852335
ht3
Allelic
Composition		 Slc25a13tm1Lct/Slc25a13+
Genetic
Background		 either: 129/Sv-Slc25a13tm1Lct or (involves: 129S1/Sv * 129X1/SvJ * CD-1)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Slc25a13tm1Lct mutation (3 available); any Slc25a13 mutation (178 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
homeostasis/metabolism
abnormal amino acid level ( J:87578 )
• 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
abnormal gluconeogenesis ( J:87578 )
• 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




Genotype
MGI:3852220
cx4
Allelic
Composition		 Gpd2tm1Tka/Gpd2tm1Tka
Slc25a13tm1Lct/Slc25a13tm1Lct
Genetic
Background		 B6.Cg-Gpd2tm1Tka Slc25a13tm1Lct
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpd2tm1Tka mutation (1 available); any Gpd2 mutation (84 available)
Slc25a13tm1Lct mutation (3 available); any Slc25a13 mutation (178 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:124766 )
• 30% fewer than expected mice are found between 20 and 40 days

homeostasis/metabolism
increased aspartic acid level ( J:124766 )
• hepatic aspartic acid levels are higher than in wild-type mice
abnormal circulating amino acid level ( J:124766 )
• 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
increased circulating ammonia level ( J:124766 )
• under fed conditions and after oral sucrose administration
increased blood urea nitrogen level ( J:124766 )
• plasma urea concentrations are higher than in wild-type mice
hypoglycemia ( J:124766 )
• compared to in wild-type mice and more severely than in Slc25a13tm1Lct homozygotes under fasting and fed conditions
decreased circulating insulin level ( J:124766 )
• compared to wild-type mice and more severely than in Gpd2tm1Tka homozygotes

liver/biliary system
hepatic steatosis ( J:124766 )
• following 16 hours of fasting

growth/size/body
decreased body weight ( J:124766 )
• by 36 days in males and 29 days in females
postnatal growth retardation ( J:124766 )

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
citrullinemia DOID:9273 J:124766




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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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Send questions and comments to User Support. 		last database update
04/23/2024
MGI 6.23 		The Jackson Laboratory