Tg(YAC72)2511Hay
Transgene Detail

Summary

• Symbol: Tg(YAC72)2511Hay
• Name: transgene insertion 2511, Michael Hayden
• MGI ID: MGI:2429756
• Synonyms: YAC72
• Transgene: Tg(YAC72)2511Hay Location: unknown
• Alliance: Tg(YAC72)2511Hay page

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(YAC72)2511Hay expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  HTT (3064)

  Htt	(MGI:96067)

• Mutation details: A YAC containing a full-length human huntingtin gene was modified with a 72 CAG repeat expansion in exon 1. The resulting construct (YAC72) was used to generate transgenic mice. The endogenous human HDH promoter directed expression in many tissues with levels in the brain and testes being the highest. (J:55405)

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: 1 strain available Cell Lines: 0 lines available

Notes

Homozygous transgenic mice are viable and fertile. In these mice the human huntingtin transgenic protein is expressed widely in many tissues (identical to the endogenous huntingtin protein), with highest expression levels in the brain and testes. Electrophysiological abnormalities can be measured by 6 months. A behavioral phenotype is first detected at 7 months when evidence of mild hyperkinetic movement disorder is noticeable. This disorder is characterized by progressive spontaneous hyperactivity during the dark phase of open field-testing. By 12 months of age selective degeneration of medium spiny neurons in the lateral striatum is observed. This degeneration is associated with the translocation of N-terminal huntingtin fragments to the nucleus.

References

• Original: J:55405 Hodgson JG, et al., A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron. 1999 May;23(1):181-92
• All: 23 reference(s)