Tg(Pcp2-ATXN1*82Q)R2Horr
Transgene Detail

Summary

• Symbol: Tg(Pcp2-ATXN1*82Q)R2Horr
• Name: transgene insertion R2, Harry T Orr
• MGI ID: MGI:6113813
• Transgene: Tg(Pcp2-ATXN1*82Q)R2Horr Location: unknown
• Alliance: Tg(Pcp2-ATXN1*82Q)R2Horr page

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(Pcp2-ATXN1*82Q)R2Horr expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  ATXN1 (6310)

  Atxn1

  (MGI:104783)

  expresses a human mutant cDNA sequence with 82 CAG repeats

• Tg(Pcp2-ATXN1*82Q)R2Horr expression driven by 1 gene
  Transgene expression driven by:
  

  Organism	Driver Gene	Note
  mouse	Pcp2 (MGI:97508)

• Mutation details: The transgene consists of a mouse Pcp2 promoter and a human mutant SCA1 cDNA sequence with 82 CAG repeats. The 835 bp fragment of mouse Pcp2 regulatory sequence include exon 1, intron 1, and part of exon 2 of Pcp2. The Pcp2 promoter targets transgene expression to Purkinje cells. Line R2 was generated. (J:261279)

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:261279 Rousseaux MWC, et al., ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism. Neuron. 2018 Mar 21;97(6):1235-1243.e5
• All: 2 reference(s)