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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
Strain Information
Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Mating System Homozygote x Homozygote (Female x Male) 30-APR-08 Species laboratory mouse Generation N13F10 (09-MAR-11)
Generation DefinitionsDonating Investigator Jie Shen, Harvard Med Sch/Brigham Women's Hosp 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.
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.
Control Information
| Control | ||
|---|---|---|
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Related Strains
Parkinson's Disease Models
View Parkinson's Disease Models (99 strains)
Additional Web Information
Visit the Parkinson's Disease Resource site for helpful information on Parkinson's and research resources.
Phenotype
Phenotype Information
Parkinson Disease 7, Autosomal Recessive Early-Onset; PARK7 - Models with phenotypic similarity to human disease where etiologies involve orthologs.1
1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s). 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
- brain histology reveals no abnormal morphology in substantia nigra, striatum or astrocytes in 24-27 month old mice (MGI Ref ID J:134518)
- dopaminergic or noradrenergic neuron loss is not detected (MGI Ref ID J:134518)
- striatal dopamine levels are similar to control (MGI Ref ID J:134518)
- no inclusions are detected in the substantia nigra or in noradrengeric neurons of the locus coeruleus (MGI Ref ID J:134518)
- abnormal action potential
- nigral neurons (dopaminergic neurons) exhibited increased action potential frequency in response to dopamine, indicating a much shorter response to dopamine than in wildtype (MGI Ref ID J:98436)
- abnormal neurotransmitter uptake
- evoked dopamine overflow in the striatum was reduced, primarily as a result of increased dopamine uptake, however had normal numbers of nigral dopaminergic neurons (MGI Ref ID J:98436)
- absent long term depression
- long term depression was absent in medium spiny neurons, however long term potentiation and cortically-evoked excitatory postsynaptic potentials were normal (MGI Ref ID J:98436)
- behavior/neurological phenotype
- *normal* behavior/neurological phenotype
- performance on the rotarod and in acoustic startle response is similar to control (MGI Ref ID J:134518)
- abnormal locomotor activation
- 18-25 month old mice exhibit decreased horizontal activity in an open field test as compared to controls (MGI Ref ID J:134518)
- abnormal stationary movement
- 18-25 month old mice exhibit fewer instances of stereotyped behavior in an open field test as compared to controls (MGI Ref ID J:134518)
- bradykinesia
- marked reduction in horizontal activity and the time spent moving and had fewer instances of stereotyped behavior at 3 months of age (MGI Ref ID J:98436)
This mouse can be used to support research in many areas including:
Neurobiology Research
Parkinson's Disease
Park7 (DJ-1) mutants
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Park7tm1Shn | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, Jie Shen | ||
| Allele Type | Targeted (knock-out) | ||
| Common Name(s) | DJ-1-; | ||
| Strain of Origin | (C57BL/6 x 129)F1 | ||
| ES Cell Line Name | Other (see notes) | ||
| Gene Symbol and Name | Park7, Parkinson disease (autosomal recessive, early onset) 7 | ||
| Chromosome | 4 | ||
| Gene Common Name(s) | CAP1; DJ-1; DJ1; SP22; | ||
| General Note | ES cell line = MKV6.5 | ||
| Molecular Note | Exon 2 was replaced with a pgk-neo cassette. Western blot failed to detect protein in mutant mice. [MGI Ref ID J:98436] | ||
Genotyping
Genotyping Information
Genotyping Protocols
Park7tm1Shn, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
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
Park7tm1Shn relatedAleyasin H; Rousseaux MW; Marcogliese PC; Hewitt SJ; Irrcher I; Joselin AP; Parsanejad M; Kim RH; Rizzu P; Callaghan SM; Slack RS; Mak TW; Park DS. 2010. DJ-1 protects the nigrostriatal axis from the neurotoxin MPTP by modulation of the AKT pathway. Proc Natl Acad Sci U S A 107(7):3186-91. [PubMed: 20133695] [MGI Ref ID J:157558]
Aron L; Klein P; Pham TT; Kramer ER; Wurst W; Klein R. 2010. Pro-survival role for Parkinson's associated gene DJ-1 revealed in trophically impaired dopaminergic neurons. PLoS Biol 8(4):e1000349. [PubMed: 20386724] [MGI Ref ID J:159857]
Kim YC; Kitaura H; Iguchi-Ariga SM; Ariga H. 2010. DJ-1, an oncogene and causative gene for familial Parkinson's disease, is essential for SV40 transformation in mouse fibroblasts through up-regulation of c-Myc. FEBS Lett 584(18):3891-5. [PubMed: 20708612] [MGI Ref ID J:164395]
Martella G; Madeo G; Schirinzi T; Tassone A; Sciamanna G; Spadoni F; Stefani A; Shen J; Pisani A; Bonsi P. 2011. Altered profile and D2-dopamine receptor modulation of high voltage-activated calcium current in striatal medium spiny neurons from animal models of Parkinson's disease. Neuroscience 177:240-51. [PubMed: 21195752] [MGI Ref ID J:170553]
Pisani A; Martella G; Tscherter A; Costa C; Mercuri NB; Bernardi G; Shen J; Calabresi P. 2006. Enhanced sensitivity of DJ-1-deficient dopaminergic neurons to energy metabolism impairment: role of Na+/K+ ATPase. Neurobiol Dis 23(1):54-60. [PubMed: 16624565] [MGI Ref ID J:111114]
Shtifman A; Zhong N; Lopez JR; Shen J; Xu J. 2011. Altered Ca2+ homeostasis in the skeletal muscle of DJ-1 null mice. Neurobiol Aging 32(1):125-32. [PubMed: 19683835] [MGI Ref ID J:168285]
Usami Y; Hatano T; Imai S; Kubo S; Sato S; Saiki S; Fujioka Y; Ohba Y; Sato F; Funayama M; Eguchi H; Shiba K; Ariga H; Shen J; Hattori N. 2011. DJ-1 associates with synaptic membranes. Neurobiol Dis 43(3):651-62. [PubMed: 21645620] [MGI Ref ID J:176995]
Yamaguchi H; Shen J. 2007. Absence of dopaminergic neuronal degeneration and oxidative damage in aged DJ-1-deficient mice. Mol Neurodegener 2:10. [PubMed: 17535435] [MGI Ref ID J:134518]
Health & husbandry
Health & Colony Maintenance Information
Animal Health Reports
Room Number AX11
Colony Maintenance
Breeding & Husbandry When 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. Mating System Homozygote x Homozygote (Female x Male) 30-APR-08 Diet Information LabDiet® 5K52/5K67
Purchasing information
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
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Live Mice
Price (US dollars $) Gender Genotypes Provided Individual Mouse $172.00 Female or Male Homozygous for Park7tm1Shn
Pairs /Price (US dollars $) Pair Genotype $344.00 Homozygous for Park7tm1Shn x Homozygous for Park7tm1Shn Standard Supply
Repository-Live. The Repository Strains represent an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. We treat orders for these strains as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
| Pricing for International shipping destinations |
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Live Mice
Price (US dollars $) Gender Genotypes Provided Individual Mouse $223.60 Female or Male Homozygous for Park7tm1Shn
Pairs /Price (US dollars $) Pair Genotype $447.20 Homozygous for Park7tm1Shn x Homozygous for Park7tm1Shn Standard Supply
Repository-Live. The Repository Strains represent an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. We treat orders for these strains as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
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|
|
Standard Supply
Repository-Live. The Repository Strains represent an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. We treat orders for these strains as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
General Supply Notes
- This strain is included in the Induced Mutant Resource Colony collection.
Control Information
| Control | ||
|---|---|---|
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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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.Ordering Information
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