Strain Name:

PN/nBSwUmabJ

Stock Number:

005052

Availability:

Repository-Cryopreserved

Description

Strain Information

Former Names PN/HandwergerJ    (Changed: 15-DEC-04 )
Type Inbred Strain;
Additional information on Inbred Strains.
Specieslaboratory mouse
H2 Haplotypeq

Appearance
albino associated genotype: presumed
Related Genotype: Tyrc/Tyrc

Important Note
This strain is homozygous for Gnat2cpfl3, cone photoreceptor function loss 3, which affects bright light (photopic) vision.

Description
PN/nBSwUmabJ mice develop a predominantly female lupus-like syndrome with age (Walker et al., 1978). Characteristics of the systemic lupus erythemoatosus syndrome include: abnormalities in B and T cell function, hypergammaglobulinemia, antibody production against multiple autoantigens, severe systemic perivasculitis and vasculitis, and immune complex-mediated glomerulonephritis (Walker et al.,1978, Luzina et al., 1999, Davidson et al., 1982, Handwerger et al.,1999). Perivascular and vascular infiltrates are composed of unusual phenotypic markers characteristic of both T and B cells (Luzina et al, 1999). By three months of age 88-100% of mice have IgG autoantibodies (Handwerger et al., 1999). Death usually occurs earlier in females as a result of glomerulonephritis and arteritis. The disease progression is accelerated in the female offspring of NZB and PN F1 crosses (Walker et al., 1986). PN/nBSwUmabJ mice develop hearing loss and otic capsule sclerosis demonstrating some parallels to human cochlear autoimmune osteogenesis (Khan et al., 2000).

Development
The inbred strain, PN/nBSwUmabJ, was developed from albino pet shop mice in1948 at Palmerston North Hospital in New Zealand. Wigley began to inbreed the colony in 1964, selecting for positive ANA (anti-nuclear antibody) in the first three generations. The primary breeding line was named PN/nA. At F9 a subline was created named PN/nB. Both lines exhibited similar characteristics of autoimmune disease (Walker et al 1978). The nA line was discarded in 1975. The nB colony was transferred to Walker (Sw) at the University of Michigan in the 1980's and then to Handwerger at the University of Maryland (Umab). The Jackson Laboratory imported the strain in 2004.

Related Strains

Strains carrying   Gnat2cpfl3 allele
002448   129S1/SvImJ
003072   ALS/LtJ
006180   CD10/JlsJ
002746   SENCARA/PtJ
002747   SENCARB/PtJ
002748   SENCARC/PtJ
006135   STOCK Sgk3fz-ica/McirJ
003773   STOCK Tg(CAG-ECFP)CK6Nagy/J
005645   STOCK Tg(CAG-mRFP1)1F1Hadj/J
004623   STOCK Tg(Fos-lacZ)34Efu/J
005667   STOCK Tg(Neurog3-cre)C1Able/J
003262   STOCK Tg(Trp53A135V)L3Ber/J
005104   STOCK Tg(tetO-HIST1H2BJ/GFP)47Efu/J
005699   STOCK Tg(tetO-Ipf1,EGFP)956.6Macd/J
View Strains carrying   Gnat2cpfl3     (14 strains)

Additional Web Information

Genetic Quality Control Annual Report

Phenotype

Phenotype Information

Research Applications

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

Immunology and Inflammation Research
Autoimmunity (lupus erythematosus)

Sensorineural Research
Vestibular and Hearing Defects

Genes & Alleles

Gene & Allele Information

Allele Symbol Gnat2cpfl3
Allele Name cone photoreceptor function loss 3
Common Name(s) Gnat2;
Strain of Originvarious
Gene Symbol and Name Gnat2, guanine nucleotide binding protein, alpha transducing 2
Chromosome 3
Gene Common Name(s) ACHM4; AW490837; GNATC; Gnat-2; Gt-2; Tcalpha; expressed sequence AW490837;
General Note This allele has been detected in the following strains either by genotyping or complementation testing: ALS/LtJ, SENCARA/PtJ, SENCARB/PtJ, SENCARC/PtJ, PN/nBSwUmabJ. (J:122428)

Phenotypic Similarity to Human Syndrome in Orthologous Human Gene: OMIM +139340 ACHROMATOPSIA 4.

Molecular Note A single nucleotide substitution of G to A at position 598 in exon 6. This mutation converts codon 200 from apartic acid to asparagine. [MGI Ref ID J:122428]

Genotyping

Genotyping Information

This strain will not have a genotyping protocol or one is not currently available.

Helpful Links

Optimizing PCR Protocols

References

References

Additional References

Chang B; Dacey MS; Hawes NL; Hitchcock PF; Milam AH; Atmaca-Sonmez P; Nusinowitz S; Heckenlively JR. 2006. Cone photoreceptor function loss-3, a novel mouse model of achromatopsia due to a mutation in Gnat2. Invest Ophthalmol Vis Sci 47(11):5017-21. [PubMed: 17065522]  [MGI Ref ID J:122428]

Davidson WF. 1982. Immunologic abnormalities of the autoimmune mouse, Palmerston North. J Immunol 129(2):751-8. [PubMed: 6282970]  [MGI Ref ID J:25382]

Walker SE. 1988. Defective primary and secondary IgG responses to thymic-dependent antigens in autoimmune PN mice. J Immunol 140(10):3426-33. [PubMed: 3283233]  [MGI Ref ID J:26163]

Walker SE; Gray RH; Fulton M; Wigley RD; Schnitzer B. 1978. Palmerston North mice, a new animal model of systemic lupus erythematosus. J Lab Clin Med 92(6):932-45. [PubMed: 310856]  [MGI Ref ID J:25377]

Walker SE; Hewett JE. 1984. Responses to T-cell and B-cell mitogens in autoimmune Palmerston North and NZB/NZW mice. Clin Immunol Immunopathol 30(3):469-78. [PubMed: 6607803]  [MGI Ref ID J:26452]

Gnat2cpfl3 related

Alexander JJ; Umino Y; Everhart D; Chang B; Min SH; Li Q; Timmers AM; Hawes NL; Pang JJ; Barlow RB; Hauswirth WW. 2007. Restoration of cone vision in a mouse model of achromatopsia. Nat Med 13(6):685-7. [PubMed: 17515894]  [MGI Ref ID J:121897]

Chang B; Dacey MS; Hawes NL; Hitchcock PF; Milam AH; Atmaca-Sonmez P; Nusinowitz S; Heckenlively JR. 2006. Cone photoreceptor function loss-3, a novel mouse model of achromatopsia due to a mutation in Gnat2. Invest Ophthalmol Vis Sci 47(11):5017-21. [PubMed: 17065522]  [MGI Ref ID J:122428]

Nusinowitz S; Ridder WH rd; Ramirez J. 2007. Temporal response properties of the primary and secondary rod-signaling pathways in normal and Gnat2 mutant mice. Exp Eye Res 84(6):1104-14. [PubMed: 17408617]  [MGI Ref ID J:126462]

Umino Y; Solessio E; Barlow RB. 2008. Speed, spatial, and temporal tuning of rod and cone vision in mouse. J Neurosci 28(1):189-98. [PubMed: 18171936]  [MGI Ref ID J:131050]

Health & husbandry

Health & Colony Maintenance Information

Currently there no information available for this strain. This may be due to the supply level of this strain.

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Pricing for USA, Canada and Mexico shipping destinations             View   International   Pricing
Weeks of AgePrice*Gender
Cryorecovery Fee $1900.00
Cryopreserved Embryos Fee $1600.00
*Price(s) in US dollars ($)

Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes
  • Cryopreserved Embryos
    This strain is also available as cryopreserved embryos from our Repository. Orders for cryopreserved embryos are supplied subject to a signed agreement that must be returned to the Customer Service Department after order placement. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos from our repository, please visit our Cryopreserved Embryos web page.
  • Cryorecovery - Standard.
    The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845.

  • This strain is included in the Mouse Mutant Resource collection.
  • Genomic DNA is available for this strain from the Mouse DNA Resource.

Pricing for International shipping destinations             View   USA, Canada and Mexico   Pricing
Weeks of AgePrice*Gender
Cryorecovery Fee $2470.00
Cryopreserved Embryos Fee $2080.00
*Price(s) in US dollars ($)

Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes
  • Cryopreserved Embryos
    This strain is also available as cryopreserved embryos from our Repository. Orders for cryopreserved embryos are supplied subject to a signed agreement that must be returned to the Customer Service Department after order placement. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos from our repository, please visit our Cryopreserved Embryos web page.
  • Cryorecovery - Standard.
    The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845.

  • This strain is included in the Mouse Mutant Resource collection.
  • Genomic DNA is available for this strain from the Mouse DNA Resource.

General Terms and Conditions

View JAX® Mice & Services Conditions of Use.

The Jackson Laboratory's Genotype Promise

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 and Purchasing Information

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Contact Information
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Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
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