Tg(Prnp-ATXN3*148Q)148.19Olri
Transgene Detail

Summary

• Symbol: Tg(Prnp-ATXN3*148Q)148.19Olri
• Name: transgene insertion 148.19, Olaf Riess
• MGI ID: MGI:5516452
• Transgene: Tg(Prnp-ATXN3*148Q)148.19Olri Location: unknown
• Alliance: Tg(Prnp-ATXN3*148Q)148.19Olri page

Transgene origin

• Strain of Origin: C57BL/6N

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(Prnp-ATXN3*148Q)148.19Olri expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  ATXN3 (4287)

  Atxn3

  (MGI:1099442)

• Mutation details: The mouse prion promoter (containing 1140 bp of the prion protein gene upstream of exon 1, the complete exon 1, intron 1, and the first 52 bp of exon 2) drives the expression of the full-length human ATAXIN 3 (ATAXIN-3c isoform) containing 148 CAG repeats. Three lines were created but only one, 148.19, produced offspring. (J:122993)

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:122993 Bichelmeier U, et al., Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence. J Neurosci. 2007 Jul 11;27(28):7418-28
• All: 3 reference(s)