Tg(Prnp-ATN1)124Dbo
Transgene Detail

Summary

• Symbol: Tg(Prnp-ATN1)124Dbo
• Name: transgene insertion 124, David R Borchelt
• MGI ID: MGI:3831350
• Synonyms: AT-FL-65Q-124
• Transgene: Tg(Prnp-ATN1)124Dbo Location: unknown
• Alliance: Tg(Prnp-ATN1)124Dbo page

Transgene origin

• Strain of Origin: (C3H x C57BL/6J)F2

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(Prnp-ATN1)124Dbo expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  ATN1 (1822)

  Atn1	(MGI:104725)

  The gene contains 65 CAG repeats.

• Mutation details: The transgene comprises a cDNA encoding full length human atrophin 1 containing a 65 residue polyglutamine tract, encoded by 65 CAG repeats, inserted into an expression cassette containing the promoter, 5' intronic and 5' and 3' untranslated region sequences of the mouse prion protein gene. This transgenic line carries more than ten copies of the construct and, as shown by northern and western blot analyses, expresses human atrophin 1 mRNA and protein in the brain. (J:70362)
• Inheritance: Dominant

Disease models

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 1 strain available Cell Lines: 0 lines available

Notes

Six independent transgenic mouse lines were generated (low expression: 79, 82, 150; high expression: 124, 125, 165) as determined by Northern blot analysis (J:70362). All lines exhibit a similar phenotype, however, high expressing lines develop symptoms beginning at 1-2 months. Low expressing lines develop symptoms at 8-12 months.

References

• Original: J:70362 Schilling G, et al., Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. Neuron. 1999 Sep;24(1):275-86
• All: 1 reference(s)