Tg(CMV-ATXN3*135Q)CPama
Transgene Detail

Summary

• Symbol: Tg(CMV-ATXN3*135Q)CPama
• Name: transgene insertion C, Patricia Maciel
• MGI ID: MGI:5646300
• Synonyms: CMVMJD135
• Transgene: Tg(CMV-ATXN3*135Q)CPama Location: unknown
• Alliance: Tg(CMV-ATXN3*135Q)CPama page

Transgene origin

• Strain of Origin: C57BL/6

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(CMV-ATXN3*135Q)CPama expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  ATXN3 (4287)

  Atxn3

  (MGI:1099442)

• Mutation details: The human ATXN3c cDNA variant carrying a repeat tract with the sequence (CAG)2CAAAAGCAGCAA(CAG)129, coding for 135 glutamines, is under the regulation of the CMV promoter. This line has two copies of the transgene and expresses the human mRNA at near-endogenous levels in the cerebellum, brainstem, forebrain and spinal cord. With breeding, mice show intergenerational CAG repeat length variation, mostly towards contraction. (J:222991)

Disease models

Expression

In Structures Affected by this Mutation:

6 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:222991 Silva-Fernandes A, et al., Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease. Neurotherapeutics. 2014 Apr;11(2):433-49
• All: 15 reference(s)