Tg(NFH)120Jpj
Transgene Detail

Summary

• Symbol: Tg(NFH)120Jpj
• Name: transgene insertion 120, Jean-Pierre Julien
• MGI ID: MGI:2182067
• Transgene: Tg(NFH)120Jpj Location: unknown
• Alliance: Tg(NFH)120Jpj page

Transgene origin

• Strain of Origin: Not Specified

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(NFH)120Jpj expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  NEFH (4744)

  Nefh	(MGI:97309)

• Mutation details: The complete human NFH gene including 9.6 kb of 5' sequences and 13.4 kb of 3' sequences was used for the transgene. Expression of the human gene in transgenic animals was confirmed by Western blot analysis. (J:69180)

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

Notes

Founder 120 is representative of 2 founders carrying 2 and 3 copies of the transgene. These animals express approximately 50% the amount of endogenous mRNA and exhibit neurological anomalies at 6 months of age.

The phenotype is more severe in homozygous mice verses hemizygous mice, implying a correlation between phenotype and expression levels of NEFH.

References

• Original: J:69180 Cote F, et al., Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis. Cell. 1993 Apr 9;73(1):35-46
• All: 1 reference(s)