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| Nomenclature |
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Symbol:
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Tg(tetO-DBT)A1Geh
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Name:
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transgene insertion A, Gregg E Homanics
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MGI ID: |
MGI:3704903 |
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Synonyms: |
TRE-E2 (line A) |
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Transgene:
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Tg(tetO-DBT)A1Geh
Location:
unknown
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Transgene
origin |
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Strain of Origin:
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C57BL/6J
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Transgene
description |
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Transgene
Type: |
Transgenic (random, expressed) |
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Mutation: |
Insertion |
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The TRE-E2 (or tetO-DBT) transgene contains a tetracycline response element and minimal human CMV promoter controlling a human E2 subunit (DBT gene) cDNA which has been modified to contain a 4x alanine linker followed by a c-myc epitope tag at the carboxy terminus to facilitate detection. Levels of the human E2 protein are approximately equal to mouse E2 protein in livers of control mice. However, when crossed to animals that express a transgene encoding the tetracycline regulated transactivator protein (Tg(tTALap)5Bjd) and are homozygous for a knockout allele of mouse E2 (Dbttm1Geh) are treated with doxycycline, the E2 protein levels correlate with only ~5-6% of normal branched-chain keto acid dehydrogenase (BCKDH) activity. It is one hypothesis that addition of the c-myc tag interferes with BCKDH subunit assembly (ie. interaction the human E2 subunit with mouse E1 and E3 complexes), thus reducing enzymatic activity. (J:119973) |
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| Find Mice (IMSR) |
Mouse strains and cell lines available from the
International Mouse Strain Resource
(IMSR)
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Phenotype
summary
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Phenotype Summary by Mammalian Phenotype terms
(show or
hide all annotated terms)
Genotypes are listed in the next section.
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Key:
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| hm |
homozygous |
ht |
heterozygous |
| cn |
conditional genotype |
cx |
complex: > 1 genome feature |
| tg |
involves transgenes |
ot |
other: hemizygous, indeterminate,... |
| N |
normal phenotype |
 |
expected model not found |
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Phenotypic
data by
genotype
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Phenotypic Data by Genotype
(show or
hide all phenotypic details)
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| Genotype | Allelic Composition | Genetic Background |
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  tg1
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Tg(tetO-DBT)A1Geh/0 |
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J |
phenotype not analyzed |
| Genotype | Allelic Composition | Genetic Background |
|
tg2
Disease Model |
Dbttm1Geh/Dbttm1Geh
Tg(tetO-DBT)A1Geh/0
Tg(tTALap)5Bjd/0 |
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB * NMRI |
lethality/postnatal life span/aging homeostasis/metabolism 1Models involving transgenes or other mutation types may also appear in other sections of the table.
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Disease
models
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1Models involving transgenes or other mutation types may also appear in other sections of the table.
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| References |
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Original: |
J:119973
Homanics GE et al.,
"Production and characterization of murine models of classic and intermediate maple syrup urine disease."
BMC Med Genet 2006;7():33
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All: |
1 reference(s)
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