Tg(tetO-DBT)525AGeh
Transgene Detail

Summary

• Symbol: Tg(tetO-DBT)525AGeh
• Name: transgene insertion 525A, Gregg E Homanics
• MGI ID: MGI:3704919
• Synonyms: TRE-E2 (line 525A)
• Transgene: Tg(tetO-DBT)525AGeh Location: unknown
• Alliance: Tg(tetO-DBT)525AGeh page

Transgene origin

• Strain of Origin: C57BL/6J

Transgene description

• Transgene Type: Transgenic (Epitope tag, Humanized sequence, Hypomorph, Inducible, Inserted expressed sequence)
• Inducer: doxycycline/tetracycline
• Mutation: Insertion
• Tg(tetO-DBT)525AGeh expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  DBT (1629)

  Dbt	(MGI:105386)

• Mutation details: The 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 and an SV40 late polyadenylation sequence to increase mRNA stability. 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 (Dbt^tm1Geh) 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 the addition of the c-myc tag interferes with BCKDH subunit assembly (ie. interaction of the human E2 subunit with mouse E1 and E3 complexes), thus reducing enzymatic activity. (J:119973)

Disease models

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 0 strains available Cell Lines: 0 lines available

References

• 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
• All: 1 reference(s)