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| Nomenclature |
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Symbol:
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Tg(SMN2)2Hung
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Name:
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transgene insertion 2, Hung Li
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MGI ID: |
MGI:3056903 |
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Synonyms: |
SMN2+ |
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Transgene:
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Tg(SMN2)2Hung
Location:
unknown
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Transgene
origin |
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Transgene
description |
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Transgene
Type: | |
Transgenic (random, expressed) |
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Mutation: | |
Insertion |
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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)
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Phenotypes
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View phenotypes for all genotypes (concatenated display).
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Disease models
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| Find Mice (IMSR) |
Mouse strains and cell lines available from the
International Mouse Strain Resource
(IMSR)
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Notes |
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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| References |
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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
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All: |
18 reference(s)
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Analysis Tools
