Tg(Prnp-ATXN3*79Q)#Hlw
Transgene Detail

Summary

• Symbol: Tg(Prnp-ATXN3*79Q)#Hlw
• Name: transgene insertion, Hung-Li Wang
• MGI ID: MGI:4940057
• Synonyms: Ataxin-3-Q79
• Transgene: Tg(Prnp-ATXN3*79Q)#Hlw Location: unknown
• Alliance: Tg(Prnp-ATXN3*79Q)#Hlw page

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(Prnp-ATXN3*79Q)#Hlw expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  ATXN3 (4287)

  Atxn3

  (MGI:1099442)

• Mutation details: The mouse promoter drive neuronal expression of the HA-tagged human cDNA with a trinucleotide repeat that results in a 79 glutamine stretch. This amplification of the glutamine tract is associated with Spinocerebellar Ataxia 3 (SCA3) in humans. Two lines with more than five copies of the transgene were generated. The pound symbol (#) denotes when no line number is specified and/or lines are pooled. (J:138381)

Disease models

Expression

In Structures Affected by this Mutation:

1 anatomical structure(s)

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:138381 Chou AH, et al., Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation. Neurobiol Dis. 2008 Jul;31(1):89-101
• All: 3 reference(s)