Strain Name:

C.Cg-Pstpip2cmo/J

Stock Number:

002864

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Availability:

Cryopreserved - Ready for recovery

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Former Names BALB/cAnPt-cmo/J    (Changed: 15-DEC-04 )
Type Congenic; Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
H2 Haplotyped

Appearance
albino, unaffected
Related Genotype: A/A Tyrp1b/Tyrp1b Tyrc/Tyrc Pstpip2cmo/+ or +/+

albino, affected
Related Genotype: A/A Tyrp1b/Tyrp1b Tyrc/Tyrc Pstpip2cmo/Pstpip2cmo

Description
Mice homozygous for the cmo spontaneous mutation are viable and fertile and live a normal life span. Homozygous mutant mice of both sexes exhibit multiple sites of inflammation in the bone. By 6-8 weeks of age kinks or swellings in the tail are evident. Hind and fore paws become swollen and red with severe deformity by three months of age. The phenotype of this mouse resembles human chronic recurrent multiple osteomyelitis.

Development
The chronic multifocal osteomyelitis mutation was first identified by Byrd et al. on their C.D2-Qa2+ congenic strain, then at N6F15. This congenic strain came from backcrossing Qa2 from DBA/2N onto BALB/cAnPt. BALB/cAnPt differs at H2-Qa2 from other sublines of BALB/c, which share the H2d haplotype with DBA/2. In 1996 N13F7 mice homozygous for cmo were sent from Dr. Michael Potter to The Jackson Laboratory where homozygotes were bred together to generate embryos for cryopreservation.

Control Information

  Control
   See control note: The approximate control BALB/cAnLil is no longer distributed by The Jackson Laboratory.
   001026 BALB/cByJ (approximate)
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
Models with phenotypic similarity to human diseases where etiology is unknown or involving genes where ortholog is unknown.
Chronic Recurrent Multifocal Osteomyelitis; CRMO
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Pstpip2cmo/Pstpip2cmo

        C.D2-Qa2
  • mortality/aging
  • *normal* mortality/aging
    • despite chronic inflammation of the extremities, mice have a normal life span and are fertile   (MGI Ref ID J:1534)
  • limbs/digits/tail phenotype
  • abnormal autopod morphology
    • hindfeet become red and swollen and are severely deformed by 3 months of age, forefeet are also affected at a lesser extent   (MGI Ref ID J:1534)
  • kinked tail
    • tail kinks evident by 6-8 weeks of age   (MGI Ref ID J:1534)
  • immune system phenotype
  • chronic inflammation
    • inflammation of the extremities   (MGI Ref ID J:1534)
    • phenotype is also penetrant when mice are crossed to BALB/c and CLA mice   (MGI Ref ID J:1534)

Pstpip2cmo/Pstpip2cmo

        C.Cg-Pstpip2cmo/J
  • limbs/digits/tail phenotype
  • abnormal autopod morphology
    • hindfeet are severely deformed by 4-6 weeks of age   (MGI Ref ID J:104476)
  • kinked tail
    • tail kinks evident by 6-8 weeks of age   (MGI Ref ID J:104476)
  • hearing/vestibular/ear phenotype
  • abnormal outer ear morphology
    • half of older mice develop erythematous, thickened outer ears with evidence of cartilage destruction   (MGI Ref ID J:104476)
  • ear inflammation
    • dermis and epidermis of outer ear is infiltrated with an inflammatory mediators including polymorphonuclear cells   (MGI Ref ID J:104476)
    • some neutrophilic microabcesses are observed   (MGI Ref ID J:104476)
  • immune system phenotype
  • abnormal lymph node morphology
    • lymph nodes contain large populations of transformed cells with features of immunoblasts   (MGI Ref ID J:104476)
  • ear inflammation
    • dermis and epidermis of outer ear is infiltrated with an inflammatory mediators including polymorphonuclear cells   (MGI Ref ID J:104476)
    • some neutrophilic microabcesses are observed   (MGI Ref ID J:104476)
  • enlarged spleen
    • approximately 1.5 times the size of controls   (MGI Ref ID J:104476)
  • osteomyelitis
    • tail sections reveal areas of dead bone surrounded by inflammatory infiltrate, predominantly neutrophils   (MGI Ref ID J:104476)
    • spinal and caudal vertebrae are nearly destroyed with a mixed inflammatory infiltrate   (MGI Ref ID J:105410)
    • this phenotype is progressive with age and begins at 3-4 weeks of age in distal vertebrae and develops in proximal vertebrae later   (MGI Ref ID J:105410)
    • no abnormalities are seen in the long bones of the limbs   (MGI Ref ID J:105410)
  • hematopoietic system phenotype
  • enlarged spleen
    • approximately 1.5 times the size of controls   (MGI Ref ID J:104476)
  • extramedullary hematopoiesis
    • evidence of extramedullary hematopoiesis in the red pulp of the spleen; may account for increased size of spleen   (MGI Ref ID J:104476)
  • skeleton phenotype
  • osteomyelitis
    • tail sections reveal areas of dead bone surrounded by inflammatory infiltrate, predominantly neutrophils   (MGI Ref ID J:104476)
    • spinal and caudal vertebrae are nearly destroyed with a mixed inflammatory infiltrate   (MGI Ref ID J:105410)
    • this phenotype is progressive with age and begins at 3-4 weeks of age in distal vertebrae and develops in proximal vertebrae later   (MGI Ref ID J:105410)
    • no abnormalities are seen in the long bones of the limbs   (MGI Ref ID J:105410)
  • craniofacial phenotype
  • abnormal outer ear morphology
    • half of older mice develop erythematous, thickened outer ears with evidence of cartilage destruction   (MGI Ref ID J:104476)
  • integument phenotype
  • deformed nails
    • thickened, dystrophic hind foot nails   (MGI Ref ID J:104476)
  • growth/size/body phenotype
  • abnormal outer ear morphology
    • half of older mice develop erythematous, thickened outer ears with evidence of cartilage destruction   (MGI Ref ID J:104476)
View Research Applications

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

Immunology, Inflammation and Autoimmunity Research
Inflammation
      osteomyelitis

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Pstpip2cmo
Allele Name chronic multifocal osteomyelitis
Allele Type Spontaneous
Common Name(s) cmo;
Strain of OriginC.D2-Qa2
Gene Symbol and Name Pstpip2, proline-serine-threonine phosphatase-interacting protein 2
Chromosome 18
Gene Common Name(s) MAYP; RGD1563090; chronic multifocal osteomyelitis; cmo;
Molecular Note Sequncing revealed a T to C transversion at nt position 293 that resulted in a highly conserved leucine to be replaced by a proline at amino acid position 98. [MGI Ref ID J:104476]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Byrd L; Grossmann M; Potter M; Shen-Ong GL. 1991. Chronic multifocal osteomyelitis, a new recessive mutation on chromosome 18 of the mouse. Genomics 11(4):794-8. [PubMed: 1686018]  [MGI Ref ID J:1534]

Hilgers J; van Nie R; Ivanyi D; Hilkens J; Michalides R; de Moes J; Poort-Keesom R; Kroezen V; von Deimling O; Kominami R; Holmes R. 1985. Genetic differences in BALB/c sublines. Curr Top Microbiol Immunol 122:19-30. [PubMed: 2994956]  [MGI Ref ID J:24314]

Pstpip2cmo related

Byrd L; Grossmann M; Potter M; Shen-Ong GL. 1991. Chronic multifocal osteomyelitis, a new recessive mutation on chromosome 18 of the mouse. Genomics 11(4):794-8. [PubMed: 1686018]  [MGI Ref ID J:1534]

Cassel SL; Janczy JR; Bing X; Wilson SP; Olivier AK; Otero JE; Iwakura Y; Shayakhmetov DM; Bassuk AG; Abu-Amer Y; Brogden KA; Burns TL; Sutterwala FS; Ferguson PJ. 2014. Inflammasome-independent IL-1beta mediates autoinflammatory disease in Pstpip2-deficient mice. Proc Natl Acad Sci U S A 111(3):1072-7. [PubMed: 24395802]  [MGI Ref ID J:206468]

Chitu V; Ferguson PJ; de Bruijn R; Schlueter AJ; Ochoa LA; Waldschmidt TJ; Yeung YG; Stanley ER. 2009. Primed innate immunity leads to autoinflammatory disease in PSTPIP2-deficient cmo mice. Blood 114(12):2497-505. [PubMed: 19608749]  [MGI Ref ID J:152809]

Chitu V; Nacu V; Charles JF; Henne WM; McMahon HT; Nandi S; Ketchum H; Harris R; Nakamura MC; Stanley ER. 2012. PSTPIP2 deficiency in mice causes osteopenia and increased differentiation of multipotent myeloid precursors into osteoclasts. Blood 120(15):3126-35. [PubMed: 22923495]  [MGI Ref ID J:192940]

Ferguson PJ; Bing X; Vasef MA; Ochoa LA; Mahgoub A; Waldschmidt TJ; Tygrett LT; Schlueter AJ; El-Shanti H. 2006. A missense mutation in pstpip2 is associated with the murine autoinflammatory disorder chronic multifocal osteomyelitis. Bone 38(1):41-7. [PubMed: 16122996]  [MGI Ref ID J:104476]

Hentunen TA; Choi SJ; Boyce BF; Dallas MR; Dallas SL; Shen-Ong GL; Roodman GD. 2000. A murine model of inflammatory bone disease Bone 26(2):183-8. [PubMed: 10678414]  [MGI Ref ID J:105410]

Lukens JR; Gross JM; Calabrese C; Iwakura Y; Lamkanfi M; Vogel P; Kanneganti TD. 2014. Critical role for inflammasome-independent IL-1beta production in osteomyelitis. Proc Natl Acad Sci U S A 111(3):1066-71. [PubMed: 24395792]  [MGI Ref ID J:206471]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

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

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2525.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3283.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   See control note: The approximate control BALB/cAnLil is no longer distributed by The Jackson Laboratory.
   001026 BALB/cByJ (approximate)
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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