Overview

Also Known As:Q58-11

In 2020, analysis performed by The Jackson Laboratory discovered Stock No. 003811 (Q58-11) does not exhibit human SCA2 RNA expression from the transgene.

Donating Investigator

Stefan M Pulst, University of Utah

Genetic overview

Genetic Background	Generation

Tg(Pcp2-SCA2)11Plt

Allele Type
Transgenic (Inserted expressed sequence, Humanized sequence)

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Research Applications

Neurobiology Research

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Details

Detailed Description

In 2020, analysis performed by The Jackson Laboratory discovered Stock No. 003811 does not exhibit human SCA2 RNA expression from the transgene. In addition, this strain has transgene copy number variations indicative of two or more insertion sites. Hemizygotes show 5-8 transgene copies (homozygotes 10-16 transgene copies).

The original characterization of these mice is listed below, but may not describe our current colony.
These transgenic mice express the human SCA2 gene under the direction of the mouse Pcp2 promoter. Homozygous mice are viable and fertile. Transcripts and protein product are detected in the cerebellum. Immunohistochemical analysis indicates that the protein product is localized to the cytoplasm. Progressive functional deficits are evident as early as eight weeks of age as measured by hind leg clasping and stride length. Also noted is a dramatic loss of Purkinje cell arbor followed by a progressive decrease in Purkinje cell number. By 24-27 weeks, the number of Purkinje cells is reduced by approximately 50%. [Huynh et al. 2000 Nature Genetics 26:44 (PMID:10973246)]

Development

A transgenic construct containing a full length SCA2 cDNA (with a 58 CAG repeat) as well as the mouse Pcp2 (Purkinje cell protein 2) promoter, untranslated and poly(A) sequences was injected into B6D2F1 pronuclei. Founder animals were obtained and crossed with B6D2F1 mice.
In 2020, analysis performed by The Jackson Laboratory discovered Stock No. 003811 has transgene copy number variations indicative of two or more insertion sites. Hemizygotes show 5-8 transgene copies (homozygotes 10-16 transgene copies).

Expression Data

Expressed Gene	SCA2, spinocerebellar ataxia 2 (olivopontocerebellar ataxia 2, autosomal dominant, ataxin 2), human
Site of Expression

Control Suggestions

Noncarrier

Additional Information

Considerations for Choosing Controls

Selected References

Huynh DP; Figueroa K; Hoang N; Pulst SM. 2000. Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human. Nat Genet 26(1):44-50PubMed: 10973246 MGI: J:93869

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Genetics

Tg(Pcp2-SCA2)11Plt

Allele Symbol: Tg(Pcp2-SCA2)11Plt

Allele Name	transgene insertion 11, Stefan M Pulst
Allele Type	Transgenic (Inserted expressed sequence, Humanized sequence)
Allele Synonym(s)	Q58-11; Tg(Pcp2SCA2)11Plt
Gene Symbol and Name	Tg(Pcp2-SCA2)11Plt, transgene insertion 11, Stefan M Pulst
Gene Synonym(s)
Promoter	Pcp2, Purkinje cell protein 2, rat
Expressed Gene	SCA2, spinocerebellar ataxia 2 (olivopontocerebellar ataxia 2, autosomal dominant, ataxin 2), human
Strain of Origin	(C57BL/6J x DBA/2J)F1
Chromosome	UN
Molecular Note	The transgene contains a full length human spinocerebellar ataxia 2 (SCA2) cDNA (with a 58 CAG repeat) as well as the mouse Pcp2 (Purkinje cell protein 2) promoter, untranslated, and poly(A) sequences. Transcripts and protein product are detected in the cerebellum.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

This mouse can be used to support research in many areas including:

Neurobiology Research
- Ataxia (Movement) Defects
  - Spinocerebellar ataxia

Mammalian Phenotype Terms by Genotype

The following phenotype relates to a compound genotype created using this strain

Genotype: Tg(Pcp2-SCA2)11Plt/Tg(Pcp2-SCA2)11Plt
involves: C57BL/6J * DBA/2J

behavior/neurological phenotype

limb grasping
- at 8 to 12 weeks of age

impaired coordination
- at 26 weeks of age, mice exhibit impaired motor performance compared to wild-type mice
- at 16 weeks of age, mice exhibit poor performance on a rotarod test compared to wild-type mice

short stride length
- at 16 weeks of age

nervous system phenotype

decreased Purkinje cell number
- by 24 to 27 weeks, mice exhibit a 50% to 53% loss of Purkinje cells compared to in wild-type mice
- at 4 weeks, mice exhibit progressive loss of calbindin-28K immunoreactive Purkinje cells that is preceded by loss of dendritic arbor unlike in wild-type mice

abnormal Purkinje cell dendrite morphology
- loss of dendritic arbor precedes loss of Purkinje cells unlike in wild-type mice

Genotype: Tg(Pcp2-SCA2)11Plt/0
involves: C57BL/6J * DBA/2J

behavior/neurological phenotype

impaired coordination
- at 26 weeks of age, mice exhibit impaired motor performance compared to wild-type mice

References

Huynh DP; Figueroa K; Hoang N; Pulst SM. 2000. Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human. Nat Genet 26(1):44-50PubMed: 10973246 MGI: J:93869

Additional - Tg(Pcp2-SCA2)11Plt related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Separated PCR:Tg(Pcp2SCA2)11Plt
- Separated PCR:Tg(Pcp2SCA2)11Plt-alternate1
- Probe:Tg(Pcp2SCA2)11Plt Probe
- QPCR:Tg(Pcp2SCA2)11Plt qPCR-alt2
- Genotyping resources and troubleshooting
Breeding Considerations

These mice originated on a B6D2F1 background. They are maintained as hemizygotes on the same background. Coat color expected from breeding is black.
- Additional Breeding and Husbandry Support
Mating System
- +/+ sibling x Hemizygote
Citation
When using the Q58-11 mouse strain in a publication, please cite the originating article(s) and include JAX stock #003811 in your Materials and Methods section.

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Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

Also Known As:Q58-11

Donating Investigator

Genetic overview

Research Applications

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Tg(Pcp2-SCA2)11Plt

Allele Symbol: Tg(Pcp2-SCA2)11Plt

Disease Terms

Research Areas By Phenotype

This mouse can be used to support research in many areas including:

Mammalian Phenotype Terms by Genotype

Genotype: Tg(Pcp2-SCA2)11Plt/Tg(Pcp2-SCA2)11Pltinvolves: C57BL/6J * DBA/2J

behavior/neurological phenotype

nervous system phenotype

Genotype: Tg(Pcp2-SCA2)11Plt/0involves: C57BL/6J * DBA/2J

behavior/neurological phenotype

References

Additional - Tg(Pcp2-SCA2)11Plt related

Genotyping Protocols

Breeding Considerations

Mating System

Citation

Strain Interest Registration

Contact Information

Interest

Terms Of Use

Licensing Information

Genotype: Tg(Pcp2-SCA2)11Plt/Tg(Pcp2-SCA2)11Plt
involves: C57BL/6J * DBA/2J

Genotype: Tg(Pcp2-SCA2)11Plt/0
involves: C57BL/6J * DBA/2J