Tg(SMN2)2Hung
Transgene Detail

Summary

• Symbol: Tg(SMN2)2Hung
• Name: transgene insertion 2, Hung Li
• MGI ID: MGI:3056903
• Synonyms: SMN2⁺
• Transgene: Tg(SMN2)2Hung Location: unknown Genetic Position: Chr4, Syntenic
• Alliance: Tg(SMN2)2Hung page

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(SMN2)2Hung expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  SMN2 (6607)

  Smn1	(MGI:109257)

• Mutation details: The transgene consists of 115kb of sequence from the SMN BAC clone 7C, including the entire human SMN2 coding region and flanking sequence. Five lines were generated. (J:59313)

Disease models

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 3 strains available Cell Lines: 0 lines available

Notes

Five lines were generated.

Hemizygous transgenic mice that are also homozygous for Smn1^tm1Hung display:

• a range of spinal muscular atrophy (SMA)-like pathologies characterized as type 1, type 2, and type 3
  • Type 1 animals do not develop hairy fur, and die before postnatal day 10
  • Type 2 animals exhibit poor activity and variable symptoms, and die at approximately 2-4 weeks
  • Type 3 animals survive to adulthood and are fertile, but have short enlarged tails

• Transgene copy number correlates with the amount of protein that contains the region encoded by exon 7 and the severity of SMA-like phenotypes in these animals

References

• Original: J:59313 Hsieh-Li HM, et al., A mouse model for spinal muscular atrophy. Nat Genet. 2000 Jan;24(1):66-70
• All: 111 reference(s)