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Strain Name:

B6.Cg-Park7tm1Shn/J

Stock Number:

006577

Availability:

Repository- Live


General Terms and Conditions

Genes & Alleles   Park7;   Park7tm1Shn;


Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Targeted Mutation
Mating SystemHomozygote x Homozygote         (Female x Male)
Specieslaboratory mouse
Donating Investigator Jie Shen,   Harvard Med Sch/Brigham Women's Hosp
GenerationN13F1 (20-DEC-07)

Strain Description
Homozygous mice are viable and fertile. Western blot analysis using antibody specific to C-terminal sequences indicates the absence of full length gene product. Homozygous mice exhibit hypokinesia and nigrostriatal dopaminergic deficits: evoked dopamine overflow in the striatum is reduced (primarily as a result of increased dopamine uptake), nigral neurons (dopaminergic neurons) have abnormal action potential characteristics, and long term depression is absent in medium spiny neurons. Also, D2-receptor mRNA abundance and radioligand binding is normal. Dopaminergic neurons from substantia nigra pars compacta (SNpc) of homozygous mice exhibit significantly higher sensitivity to energy metabolism impairment and nigral dopaminergic neurons are particularly sensitive to Na+/K+ ATPase impairment. These mutant mice may be useful in studies of Parkinson's disease, dopaminergic physiology, nigrostriatal function, locomotor inactivity, and other neurobiological research.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. Mice with this mutation were originally published on a mixed B6;129 genetic background. It should be noted that the phenotype could vary from that originally described. The strain description will be modified as published results become available.

Strain Development
A targeting vector was designed to replace exon 2 of the endogenous gene with a PGK-neomycin cassette. The construct was electroporated into the "B6/129 F1"-derived MKV6.5 embryonic stem (ES) cells. Correctly targeted ES cells were injected into blastocysts. The resulting mutant mice were backcrossed to C57BL/6 inbred mice for at least 12 generations before arriving at The Jackson Laboratory.

Related Disease (OMIM) Terms

Parkinson Disease 7, Autosomal Recessive Early-Onset; PARK7
Parkinson Disease; PD
Mammalian Phenotype Terms assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Park7tm1Shn/Park7tm1Shn

        involves: 129 * C57BL/6
  • nervous system phenotype
  • *normal* nervous system phenotype (MGI Ref ID J:134518)
    • brain histology reveals no abnormal morphology in substantia nigra, striatum or astrocytes in 24-27 month old mice
    • dopaminergic or noradrenergic neuron loss is not detected
    • striatal dopamine levels are similar to control
    • no inclusions are detected in the substantia nigra or in noradrengeric neurons of the locus coeruleus
    • abnormal action potential (MGI Ref ID J:98436)
      • nigral neurons (dopaminergic neurons) exhibited increased action potential frequency in response to dopamine, indicating a much shorter response to dopamine than in wildtype
    • abnormal neurotransmitter uptake (MGI Ref ID J:98436)
      • evoked dopamine overflow in the striatum was reduced, primarily as a result of increased dopamine uptake, however had normal numbers of nigral dopaminergic neurons
    • absent long term depression (MGI Ref ID J:98436)
      • long term depression was absent in medium spiny neurons, however long term potentiation and cortically-evoked excitatory postsynaptic potentials were normal
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype (MGI Ref ID J:134518)
    • performance on the rotarod and in acoustic startle response is similar to control
    • abnormal locomotor activation (MGI Ref ID J:134518)
      • 18-25 month old mice exhibit decreased horizontal activity in an open field test as compared to controls
      • bradykinesia (MGI Ref ID J:98436)
        • marked reduction in horizontal activity and the time spent moving and had fewer instances of stereotyped behavior at 3 months of age
      • decreased vertical activity (MGI Ref ID J:98436)
        • reduced vertical activity and time spent rearing at 3 months of age
    • abnormal stationary movement (MGI Ref ID J:134518)
      • 18-25 month old mice exhibit fewer instances of stereotyped behavior in an open field test as compared to controls
      • bradykinesia (MGI Ref ID J:98436)
        • marked reduction in horizontal activity and the time spent moving and had fewer instances of stereotyped behavior at 3 months of age

Gene & Allele Details

Allele Symbol Park7tm1Shn
Allele Name targeted mutation 1, Jie Shen
Common Name(s) DJ-1-;
Strain of Origin(C57BL/6 x 129)F1
ES Cell Line NameOther (see notes)
Gene Symbol and Name Park7, Parkinson disease (autosomal recessive, early onset) 7
Chromosome 4
Gene Common Name(s) CAP1; DJ-1; DJ1; FLJ27376; FLJ34360; FLJ92274; SP22;
General Note ES cell line = MKV6.5
Molecular Note Exon 2 was deleted upon insertion of a pgk-neo cassette. Western blot failed to detect protein in mutant mice. [MGI Ref ID J:98436]

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Genotyping Protocols

Park7tm1Shn

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, heterozygous or homozygous mice can be bred. The donating investigator maintains their colony by breeding heterozygotes with C57BL/6 inbred mice.
Diet Information LabDiet® 5K52/5K67

Additional Web Information

Congenic Nomenclature

Animal Health Reports

Room Number           AX11

Research Applications

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

Neurobiology Research
Parkinson's Disease (Park7 (DJ-1) mutants)
Parkinson's Disease

References

Selected Reference(s)

Goldberg MS; Pisani A; Haburcak M; Vortherms TA; Kitada T; Costa C; Tong Y; Martella G; Tscherter A; Martins A; Bernardi G; Roth BL; Pothos EN; Calabresi P; Shen J. 2005. Nigrostriatal dopaminergic deficits and hypokinesia caused by inactivation of the familial Parkinsonism-linked gene DJ-1. Neuron 45(4):489-96. [PubMed: 15721235]  [MGI Ref ID J:98436]

Additional References

Price and Supply Information

Strain Name: B6.Cg-Park7tm1Shn/J
Stock Number: 006577

Price Details

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Supply Details

Standard SupplyRepository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes Usually shipped between four and eight weeks of age.
This strain is included in the Induced Mutant Resource Colony collection.
LicensingSee General Terms and Conditions below for Licensing and Use Restrictions  
Control InformationView Control Information in Strain Details.

General Terms and Conditions

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- Use of MICE by companies or for-profit entities requires a license prior to shipping.

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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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