Overview

These mice develop hearing loss and otic capsule sclerosis as well as a predominantly female lupus-like syndrome.

Genetic overview

Genetic Background	Generation

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Research Applications

Sensorineural Research
Immunology, Inflammation and Autoimmunity Research

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Important Note

This strain may be homozygous for Gnat2^cpfl3, cone photoreceptor function loss 3, which affects bright light (photopic) vision.

Detailed 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.

Genetics

Gnat2^cpfl3

Allele Symbol: Gnat2^cpfl3

Allele Name	cone photoreceptor function loss 3
Allele Type	Spontaneous
Allele Synonym(s)	no cones
Gene Symbol and Name	Gnat2, guanine nucleotide binding protein, alpha transducing 2
Gene Synonym(s)
Strain of Origin	various
Chromosome	3
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) The allele has also been reported in NMRI and CD1 (Lluis Montoliu, J:212307)
Molecular Note	A single nucleotide substitution of G to A at coding nucleotide 598 in exon 6. This mutation converts codon 200 from aspartic acid to asparagine (p.D200N).

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Gnat2^cpfl3 related

Sensorineural Research
- Eye Defects

Sensorineural Research
- Hearing Defects
Immunology, Inflammation and Autoimmunity Research
- Autoimmunity
  - lupus erythematosus

Mammalian Phenotype Terms by Genotype

Phenotype Information

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):5017-21PubMed: 17065522 MGI: J:122428
Davidson WF. 1982. Immunologic abnormalities of the autoimmune mouse, Palmerston North. J Immunol 129(2):751-8PubMed: 6282970 MGI: J:25382
Walker SE. 1988. Defective primary and secondary IgG responses to thymic-dependent antigens in autoimmune PN mice. J Immunol 140(10):3426-33PubMed: 3283233 MGI: 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):932-45PubMed: 310856 MGI: 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):469-78PubMed: 6607803 MGI: J:26452

Additional - Gnat2^cpfl3 related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Appearance
- albino associated genotype: presumed
  Related Genotype: Tyr^c/Tyr^c
Citation
When using the PN/nBSwUmabJ mouse strain in a publication, please include JAX stock #005052 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
	Inbred, 1 pair minimum will be supplied

Related Products and Services

Frozen Mouse Embryo	PN/nBSwUmabJ Frozen Embryos	$2595.00
Frozen Mouse Embryo	PN/nBSwUmabJ Frozen Embryos	$2595.00
Frozen Mouse Embryo	PN/nBSwUmabJ Frozen Embryos	$3373.50
Frozen Mouse Embryo	PN/nBSwUmabJ Frozen Embryos	$3373.50

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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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

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