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Tg(Pcp2-ATXN1*82Q)5Horr
Transgene Detail
Nomenclature
Symbol: Tg(Pcp2-ATXN1*82Q)5Horr
Name: transgene insertion 5, Harry T Orr
MGI ID: MGI:2447854
Synonyms: ataxin-1-82Q, ATXN1[82Q]-S776, B02, B05, B05 SCA1, Pcp2-SCA1[82Q], PS-82, SCA1-82Q, SCA1[82Q], SCA1 B05, Tg(Pcp2-SCA1)PS82Horr
Transgene: Tg(Pcp2-ATXN1*82Q)5Horr  Location: unknown  
Transgene
origin
Strain of Origin:  FVB/N
Transgene
description
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
 
Tg(Pcp2-ATXN1*82Q)5Horr expresses 1 gene
 
Mutation detailsThe transgene consists of a mouse Pcp2 promoter and a human mutant SCA1 cDNA sequence with 82 CAG repeats. The 835 bp fragment of mouse Pcp2 regulatory sequence include exon 1, intron 1, and part of exon 2 of Pcp2. The Pcp2 promoter targets transgene expression to Purkinje cells. Transgene expression was confirmed by Northern blot analysis of cerebellar extracts. However, Western blot analysis did not detect mutant protein in any of the six transgenic lines. PCR analysis showed CAG repeat instability in some of the transgenic lines. Authors presume rearrangement of CAG repeats in some of the tandemly arrayed transgene copies occurred during integration. (J:28932)
Phenotypes
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Disease models
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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
Notes
Six transgenic lines were created carrying between 5-50 copies of the integrated transgene. All lines expressed transgene mRNA, but no protein product.

References
Original:  J:28932 Burright EN, et al., SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell. 1995 Sep 22;82(6):937-48
All:  24 reference(s)

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last database update
01/13/2015
MGI 5.21
The Jackson Laboratory