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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 A1, 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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Mutation details: 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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Phenotypes
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View phenotypes for all genotypes (concatenated display).
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Disease models
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| Find Mice (IMSR) |
Mouse strains and cell lines available from the
International Mouse Strain Resource
(IMSR)
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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: |
2 reference(s)
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Analysis Tools
