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Phenotypes Associated with This Genotype
Genotype
MGI:3704920
Allelic
Composition
Dbttm1Geh/Dbttm1Geh
Tg(tetO-DBT)525AGeh/0
Tg(Cebpb-tTA)5Bjd/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB * NMRI
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dbttm1Geh mutation (1 available); any Dbt mutation (7 available)
Tg(Cebpb-tTA)5Bjd mutation (3 available)
Tg(tetO-DBT)525AGeh mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• approximately 11% of pups surviving to weaning have this genotype, compared to a theoretical survival rate or 14%, indicating high degree of rescue of the Dbt-deficient phenotype
• only 12% (2/16) transgenic mice survive to 20 weeks; two surviving mice live to 40 and 60 weeks, respectively

homeostasis/metabolism
• blood levels of glutamate are reduced compared to controls, but greater than in Dbttm1Geh homozygotes
• BCAA/alanine (BCAA -branched-chain amino acids - leucine + isoleucine + valine) values are intermediate between controls and Dbttm1Geh homozygotes between 4-10 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
maple syrup urine disease DOID:9269 OMIM:246900
OMIM:248600
OMIM:615135
J:119973


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
03/13/2019
MGI 6.13
The Jackson Laboratory