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| Nomenclature |
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Symbol:
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Tg(Prnp-ATN1)124Dbo
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Name:
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transgene insertion 124, David R Borchelt
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MGI ID: |
MGI:3831350 |
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Synonyms: |
AT-FL-65Q-124 |
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Transgene:
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Tg(Prnp-ATN1)124Dbo
Location:
unknown
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Transgene
origin |
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Strain of Origin:
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(C3H x C57BL/6J)F2
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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 comprises a cDNA encoding full length human atrophin 1 containing a 65 residue polyglutamine tract, encoded by 65 CAG repeats, inserted into an expression cassette containing the promoter, 5' intronic and 5' and 3' untranslated region sequences of the mouse prion protein gene. This transgenic line carries more than ten copies of the construct and, as shown by northern and western blot analyses, expresses human atrophin 1 mRNA and protein in the brain. (J:70362)
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Inheritance: | |
Dominant |
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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 |
Six independent transgenic mouse lines were generated (low expression: 79, 82, 150; high expression: 124, 125, 165) as determined by Northern blot analysis (J:70362). All lines exhibit a similar phenotype, however, high expressing lines develop symptoms beginning at 1-2 months. Low expressing lines develop symptoms at 8-12 months.
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| References |
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Original: |
J:70362
Schilling G et al.,
"Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA."
Neuron 1999 Sep;24(1):275-86
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All: |
1 reference(s)
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Analysis Tools
