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Allele : Tg(SMN2)2Hung transgene insertion 2, Hung Li
Primary Identifier  MGI:3056903 Allele Type  Transgenic
Attribute String  Humanized sequence, Inserted expressed sequence Gene  Tg(SMN2)2Hung
Strain of Origin  FVB/N Is Recombinase  false
Is Wild Type  false
description  Five lines were generated.

Hemizygous transgenic mice that are also homozygous for Smn1tm1Hung 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
molecularNote  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.
  • mutations:
  • Insertion
  • synonyms:
  • SMN2<+>,
  • SMN2<+>
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1 Feature

Genome

1 Expresses

0 Mutation Involves

Phenotype

Mouse alleles --> Mammalian phenotypes (MP terms)

 

Other

5 Carried By

0 Driven By

114 Publication categories





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