Tg(SMN2)1Hung
Transgene Detail
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Symbol: |
Tg(SMN2)1Hung |
Name: |
transgene insertion 1, Hung Li |
MGI ID: |
MGI:2383995 |
Synonyms: |
SMN2 |
Transgene: |
Tg(SMN2)1Hung Location: unknown
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Alliance: |
Tg(SMN2)1Hung page
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Transgene Type: |
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Transgenic (Inserted expressed sequence) |
Mutation: |
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Insertion
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Tg(SMN2)1Hung expresses
1 gene
Transgene expresses:
Organism |
Expressed Gene |
Homolog in Mouse |
Note |
human |
SMN2 (6607) |
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Tg(SMN2)1Hung expression driven by
1 gene
Transgene expression driven by:
Organism |
Driver Gene |
Homolog in Mouse |
Note |
human |
SMN2 (6607) |
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Mutation details: A 115 kb genomic fragment from a BAC clone containing the entire human SMN2 and SERF1A genes, and part of BIRC1, was used as the transgene. Transgenic animals were identified by PCR genotype analysis.
(J:59313)
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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
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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
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Original: |
J:59313 Hsieh-Li HM, et al., A mouse model for spinal muscular atrophy. Nat Genet. 2000 Jan;24(1):66-70 |
All: |
10 reference(s) |
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