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Strain Name:

C57BL/KaLawRij-Sharpincpdm/RijSunJ

Stock Number:

007599

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General Terms and Conditions

Genes & Alleles   Sharpin;   Sharpincpdm;

Product Information

Strain Details

Type JAX® GEMM® Strain - Coisogenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Spontaneous Mutation
Mating SystemHeterozygote x Heterozygote         (Female x Male)
Specieslaboratory mouse
H2 Haplotypeb
Generation?+N1F1 (06-MAY-08)

Appearance
black
Related Genotype: a/a

Strain Description
Mice homozygous for this spontaneous mutation develop a severe, chronic, inflammatory skin disease beginning at 3-5 weeks of age. Mice appear runted and life span is shortened. In addition to dermatitis, homozygotes exhibit multi-organ inflammation with eosinophilia, defective TH1 cytokine production, splenomegaly, absent Peyer's patches (adult), diminished serum immunoglobulins, and an absence of B cell follicles, follicular dendritic cells, and germinal centers in secondary lymph organs. This mutant mouse strain may be useful in studies related to eosinophilic dermatitis, inflammation, and secondary lymphoid organ deveopment.

Strain Development
This cpdm spontaneous mutation arose in a colony of C57BL/KaLawRij held at TNO-Institute in the Netherlands. Mice from the cpdm colony were sent to Dr. John Sundberg at The Jackson Laboratory in 1993, and were maintained by sibling matings in a private research colony until they was donated to The Jackson Laboratory Repository in 2007.

Related Disease (OMIM) Terms

Dermatitis, Atopic
Mammalian Phenotype Terms assigned by genotype

Sharpincpdm/Sharpincpdm

        C57BL/KaLawRij-Sharpincpdm
  • skin/coat/nails phenotype
  • abnormal coat appearance (MGI Ref ID J:31745)
    • at 3 weeks, affected mice have thinner fur than controls
  • abnormal dermal layer morphology (MGI Ref ID J:14539)
    • in mice >28 weeks of age, dendritic cells with melanin granules are found in dermis and epidermis; heavily laden melanocytes are present in dermis
    • mixed cellular infiltration to dermis (MGI Ref ID J:14539)
      • inflammatory cells, predominantly granulocytes and macrophages
      • fewer neutrophils are observed in dermis and epidermis in aged mice (>28 weeks)
      • in excess of controls, observed from 1 week of age, and progresses to greater severity at 4 weeks
      • 24 hours after i.p. injection of tumor necrosis factor into ears, mutant ears show less infiltration by neutrophils and fewer macrophages at 6-12 weeks of age
  • abnormal epidermal layer morphology (MGI Ref ID J:14539)
    • mild spongiosis (edema) of the epidermis is seen occasionally
    • abnormal basal cell layer morphology (MGI Ref ID J:14539)
      • basal cell layer shows increased mitosis
      • thickness is increased due to cell proliferation at 4 weeks
    • abnormal cornified layer morphology (MGI Ref ID J:31745)
      • some animals display intracorneal microabcesses at 5 weeks
      • hyperkeratosis (MGI Ref ID J:14539)
        • at 4 weeks
      • parakeratosis (MGI Ref ID J:14539)
        • multifocal, observed at 4 weeks
    • abnormal granular layer morphology (MGI Ref ID J:14539)
      • layer is thicker or of normal thickness and absent beneath parakeratotic mounds in mutants
      • some degenerated granulocytes mix with parakeratotic mounds and form microabscesses in keratotic layer
    • abnormal keratinocyte morphology (MGI Ref ID J:31745)
      • thickness is increased due to cell proliferation
      • abnormal keratinocyte apoptosis (MGI Ref ID J:14539)
        • single cell death of keratinocytes in epidermis is seen
        • some apoptosis is observed starting at 3 weeks
        • 24 hours after i.p. injection of tumor necrosis factor into ears, there is marked apoptosis in epidermis of mutants at 6-12 weeks of age
    • acanthosis (MGI Ref ID J:31745)
      • observed at 4 weeks
    • epidermal hyperplasia (MGI Ref ID J:14539)
    • thickened epidermis (MGI Ref ID J:14539)
      • interfollicular epidermis is thickened (from 8.8 um to 55.6 um)
      • significantly increased at 4 weeks compared to control, showing increased cellular proliferation
  • abnormal hair follicle morphology (MGI Ref ID J:14539)
    • epidermal changes along hair shafts result in follicular keratosis
    • increased numbers of proliferating cells are found
  • abnormal hair shaft morphology (MGI Ref ID J:14539)
    • epidermal changes along hair shafts result in hair shaft degeneration
  • alopecia (MGI Ref ID J:68129)
    • clinical alopecia develops by 5 weeks of age and becomes progressively worse
  • dermatitis (MGI Ref ID J:14539)
    • mice develop dermatitis with associated skin lesions starting at 2-3 weeks of age
    • after 5 weeks of age, mice suffer from severe pruritus (itching) demonstrated by severe scratching
  • folliculitis (MGI Ref ID J:31745)
    • folliculitis with some degenerated hair shafts is observed at 3 weeks of age
  • premature hair loss (MGI Ref ID J:14539)
    • beginning at 5 weeks of age, affected mice develop hair loss
    • at 4 weeks, dorsal neck and ventral chest develop hair loss
  • reddish skin (MGI Ref ID J:14539)
    • beginning at 5 weeks of age, reddening of skin of dorsal neck and ventral chest is observed, spreading to most skin, except tail, feet, and ear pinnae by 12-15 weeks
    • at 3 weeks, reddening of the axilla is observed, such that mutants can all be identified
  • scaly skin (MGI Ref ID J:14539)
    • at 4 weeks, dorsal neck and ventral chest develop mild scaling
    • scaling skin is found by 5 weeks of age and becomes progressively worse
  • skin edema (MGI Ref ID J:14539)
    • mild spongiosis (intracellular edema) of the epidermis is seen occasionally
  • skin lesions (MGI Ref ID J:14539)
    • lesions are characterized by erythema, severe hair loss, and mild scaling at 20 weeks of age
    • lesions are similar in aged mice (28-30 weeks of age)
    • lesions progress with age, from 2-3 weeks; at 6 weeks, lesions are extensive and severe
  • immune system phenotype
  • abnormal chemokine physiology (MGI Ref ID J:116833)
    • eotaxin (CCL11) protein concentration in skin of affected homozygotes compared to wild type
  • abnormal humoral immune response (MGI Ref ID J:53575)
    • 4 weeks after immunization with DNP-KLH (DNP-keyhole limpet hemocyanin), DNP-specific antibody titers are lower in mutants than control
    • decreased immunoglobulin level (MGI Ref ID J:53575)
      • after immunization with DNP-KLH, reductions in DNP-specific IgM (2-fold), IgG3 (4-fold), IgG1 (12-fold), IgG2a (37-fold) and IgG2b (7-fold) antibodies is observed in mutants
      • decreased IgA level (MGI Ref ID J:53575)
        • total IgA is significantly lower in serum compared to controls; IgA in feces is greatly reduced at 6-12 weeks of age
      • decreased IgE level (MGI Ref ID J:31745)
        • serum levels are decreased ~10-fold compared to controls
        • total IgE is significantly lower in serum
      • decreased IgG level (MGI Ref ID J:53575)
        • total IgG is significantly lower in serum
    • increased IgM level (MGI Ref ID J:53575)
      • levels in feces are significantly increased compared to controls
  • abnormal immune system organ morphology (MGI Ref ID J:14539)
    • atrophy of lymphoid tissue with increasing age is observed
    • abnormal lymph node morphology (MGI Ref ID J:14539)
      • at >28 weeks of age, heavily laden melanocytes are present in draining lymph nodes
      • peripheral lymph nodes draining the skin are swollen, having a thin cortex without clearly defined follicles, and large numbers of eosinophils in the medulla
      • abnormal mesenteric lymph node morphology (MGI Ref ID J:53575)
        • nodes are small, with medulla and occasionally entire lymph node replaced with connective tissue
    • abnormal spleen morphology (MGI Ref ID J:53575)
      • abnormal spleen white pulp morphology (MGI Ref ID J:31745)
        • white pulp is poorly defined at 6-12 weeks of age
        • abnormal spleen B cell follicle morphology (MGI Ref ID J:53575)
          • spleens have no defined follicles and germinal centers are absent
          • absent spleen germinal center (MGI Ref ID J:53575)
        • absent spleen marginal zone (MGI Ref ID J:53575)
          • no evidence of a marginal zone is found
      • enlarged spleen (MGI Ref ID J:14539)
        • 3- to 6-fold increase in spleen size is observed
        • spleens in mutants are enlarged 3- to 5-fold due to extramedullary hematopoiesis
    • absent Peyer's patches (MGI Ref ID J:53575)
      • absent but controls have 5-7; no rudimentary PPs are found in the small intestine of mutants
    • large lymphoid organs (MGI Ref ID J:31745)
      • at age of 3 weeks, deep and superficial cervical, thoracic, axillary and brachial lymph nodes and spleen are mildly enlarged
  • decreased B cell number (MGI Ref ID J:53575)
    • numbers of B lymphocytes are slightly decreased in mutant spleen at 6-12 weeks of age
  • decreased T cell number (MGI Ref ID J:53575)
    • numbers are moderately decreased in mutant spleen
    • decreased CD4-positive T cell number (MGI Ref ID J:53575)
      • percentage of cell is significantly reduced (16.8% in control vs 8.5%)
  • dermatitis (MGI Ref ID J:14539)
    • mice develop dermatitis with associated skin lesions starting at 2-3 weeks of age
    • after 5 weeks of age, mice suffer from severe pruritus (itching) demonstrated by severe scratching
  • esophageal inflammation (MGI Ref ID J:31745)
    • lesions, lesions comparable to skin lesions, are observed starting at 4 weeks; lesions are immediately moderate to severe
  • folliculitis (MGI Ref ID J:31745)
    • folliculitis with some degenerated hair shafts is observed at 3 weeks of age
  • increased eosinophil cell number (MGI Ref ID J:14539)
    • mutants have increased counts of eosinophilic alveolar macrophages
    • eosinophil infiltration of red and white pulp of spleen is seen starting at 4 weeks of age
  • increased mast cell number (MGI Ref ID J:14539)
    • numbers are significantly increased in dermis at 6-8 weeks (171/sq. mm) and increase with age (594/sq.mm at 28-30 weeks)
    • 6 week old mice have increased mast cell numbers compared to controls (213/sq.mm vs 82/sq.mm)
  • joint inflammation (MGI Ref ID J:14539)
    • at 20 weeks of age, synovia and periarticular and peritendinous connective tissues are infiltrated by low to moderate number of macrophages and neutrophils, frequently affecting coxofemoral, tibiofemoral, and invertebral joints, and knee tendons; more common in female mice
  • lung inflammation (MGI Ref ID J:31745)
    • lesions are observed from 1 week of age; inflammatory cells are mainly eosinophils with some monocytes
  • lymph node inflammation (MGI Ref ID J:31745)
    • at 1 week of age, deep and superficial cervical, thoracic, axillary and brachial lymph nodes display acute lymphadenitis and perilymphadenitis with eosinophil infiltration of cortex and medulla, varying from mild to severe
  • stomach inflammation (MGI Ref ID J:31745)
    • lesions, lesions comparable to skin lesions but no parakeratosis, are observed starting at 4 weeks; most severe at junction of forestomach and glandular stomach, but not as severe as in esophagus
    • infliltrating eosinophils form intraepithelial and intracorneal pustules
  • hematopoietic system phenotype
  • abnormal spleen morphology (MGI Ref ID J:53575)
    • abnormal spleen white pulp morphology (MGI Ref ID J:31745)
      • white pulp is poorly defined at 6-12 weeks of age
      • abnormal spleen B cell follicle morphology (MGI Ref ID J:53575)
        • spleens have no defined follicles and germinal centers are absent
        • absent spleen germinal center (MGI Ref ID J:53575)
      • absent spleen marginal zone (MGI Ref ID J:53575)
        • no evidence of a marginal zone is found
    • enlarged spleen (MGI Ref ID J:14539)
      • 3- to 6-fold increase in spleen size is observed
      • spleens in mutants are enlarged 3- to 5-fold due to extramedullary hematopoiesis
  • decreased B cell number (MGI Ref ID J:53575)
    • numbers of B lymphocytes are slightly decreased in mutant spleen at 6-12 weeks of age
  • decreased T cell number (MGI Ref ID J:53575)
    • numbers are moderately decreased in mutant spleen
    • decreased CD4-positive T cell number (MGI Ref ID J:53575)
      • percentage of cell is significantly reduced (16.8% in control vs 8.5%)
  • extramedullary hematopoiesis (MGI Ref ID J:53575)
    • marked, accounts for splenomegaly
    • at 2 weeks of age, mutants show moderate extramedullary myelopoiesis in liver and spleen
  • increased bone marrow cell number (MGI Ref ID J:14539)
    • bone marrow is hyperplastic with extensive myelopoiesis
  • increased eosinophil cell number (MGI Ref ID J:14539)
    • mutants have increased counts of eosinophilic alveolar macrophages
    • eosinophil infiltration of red and white pulp of spleen is seen starting at 4 weeks of age
  • increased mast cell number (MGI Ref ID J:14539)
    • numbers are significantly increased in dermis at 6-8 weeks (171/sq. mm) and increase with age (594/sq.mm at 28-30 weeks)
    • 6 week old mice have increased mast cell numbers compared to controls (213/sq.mm vs 82/sq.mm)
  • growth/size phenotype
  • postnatal growth retardation (MGI Ref ID J:14539)
    • mice show moderate growth retardation with aging
  • weight loss (MGI Ref ID J:31745)
    • weight reduction is observed from age of 5 weeks (7%) and at 6 weeks reduction of 12% is observed
  • homeostasis/metabolism phenotype
  • skin edema (MGI Ref ID J:14539)
    • mild spongiosis (intracellular edema) of the epidermis is seen occasionally
  • digestive/alimentary phenotype
  • abnormal esophageal epithelium morphology (MGI Ref ID J:14539)
    • at 20 weeks, similar changes to those seen in epidermis are found, with exception of parakeratosis
    • thickness of esophageal epithelium is increased from 38.2 um to 75.4 um in mutants; less severe in aged mice
    • lesions are prominent, with spongiform pustules occurring in esophagus
    • lesions, lesions comparable to skin lesions, are observed starting at 4 weeks; lesions are immediately moderate to severe
  • abnormal stomach epithelium morphology (MGI Ref ID J:14539)
    • at 20 weeks, similar changes to those seen in epidermis are found, with exception of parakeratosis
    • thickness of forestomach epithelium is increased from 34.1 um to 68.9 um in mutants; less severe in aged mice
  • abnormal tongue squamous epithelium morphology (MGI Ref ID J:31745)
    • at 2 weeks of age, first lesions of epithelium are seen; lesions are immediately moderate to severe
  • esophageal inflammation (MGI Ref ID J:31745)
    • lesions, lesions comparable to skin lesions, are observed starting at 4 weeks; lesions are immediately moderate to severe
  • stomach inflammation (MGI Ref ID J:31745)
    • lesions, lesions comparable to skin lesions but no parakeratosis, are observed starting at 4 weeks; most severe at junction of forestomach and glandular stomach, but not as severe as in esophagus
    • infliltrating eosinophils form intraepithelial and intracorneal pustules
  • liver/biliary system phenotype
  • abnormal liver morphology (MGI Ref ID J:14539)
    • portal and perivascular areas in liver are infiltrated by neutrophils and macrophages
    • at 3 weeks, small perivascular infiltrates of eosinophils and macrophages are observed, with progressive severity
  • reproductive system phenotype
  • female infertility (MGI Ref ID J:14539)
    • females are infertile
  • reduced male fertility (MGI Ref ID J:31745)
    • males older than 11 weeks show reduced fertility
  • respiratory system phenotype
  • abnormal lung interstitium morphology (MGI Ref ID J:14539)
    • perivascular and peribronchiolar connective tissues of lung show similar, but less conspicuous infiltrations
  • lung inflammation (MGI Ref ID J:31745)
    • lesions are observed from 1 week of age; inflammatory cells are mainly eosinophils with some monocytes
  • skeleton phenotype
  • abnormal joint capsule morphology (MGI Ref ID J:14539)
    • synovial space is often dilated, containing some neutrophils and proteinaceous material
  • joint inflammation (MGI Ref ID J:14539)
    • at 20 weeks of age, synovia and periarticular and peritendinous connective tissues are infiltrated by low to moderate number of macrophages and neutrophils, frequently affecting coxofemoral, tibiofemoral, and invertebral joints, and knee tendons; more common in female mice
  • cardiovascular system phenotype
  • abnormal angiogenesis (MGI Ref ID J:14539)
    • blood vessel proliferation in dermis is observed, with tortuous dilated capillaries present in superficial dermis
  • vision/eye phenotype
  • thick eyelids (MGI Ref ID J:31745)
    • first sign of phenotypic change is thickening of eyelids, no earlier than 2 weeks of age

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

Sharpincpdm/Sharpincpdm

        involves: C57BL/KaLawRij * CAST/EiJ
  • skin/coat/nails phenotype
  • alopecia (MGI Ref ID J:68129)
    • a CAST/EiJ derived modifier results in incomplete penetrance of the skin phenotype
  • scaly skin (MGI Ref ID J:68129)
    • a CAST/EiJ derived modifier results in incomplete penetrance of the skin phenotype

Sharpincpdm/Sharpincpdm

        involves: C57BL/KaLawRij * FVB/NJ
  • immune system phenotype
  • abnormal spleen B cell follicle morphology (MGI Ref ID J:121810)
    • spleens have no defined follicles and germinal centers are absent
    • absent spleen germinal center (MGI Ref ID J:121810)
  • absent Peyer's patches (MGI Ref ID J:121810)
    • no organized follicular structure are present in the antimesenteric wall of the small intestine
  • skin inflammation (MGI Ref ID J:121810)
    • the FVB/NJ background modifies the phenotype such that the skin shows mild inflammation and scattered apoptotic keratinocytes with a delayed onset
  • skin/coat/nails phenotype
  • skin inflammation (MGI Ref ID J:121810)
    • the FVB/NJ background modifies the phenotype such that the skin shows mild inflammation and scattered apoptotic keratinocytes with a delayed onset
  • hematopoietic system phenotype
  • abnormal spleen B cell follicle morphology (MGI Ref ID J:121810)
    • spleens have no defined follicles and germinal centers are absent
    • absent spleen germinal center (MGI Ref ID J:121810)

Gene & Allele Details

Allele Symbol Sharpincpdm
Allele Name chronic proliferative dermatitis
Common Name(s) cpd; cpdm;
Strain of OriginC57BL/Ka
Gene Symbol and Name Sharpin, SHANK-associated RH domain interacting protein
Chromosome 15
Gene Common Name(s) 0610041B22Rik; AW121341; Conneck1; DKFZp434N1923; MGC93373; RIKEN cDNA 0610041B22 gene; SIPL1; chronic proliferative dermatitis; cpdm; expressed sequence AW121341;
General Note A spontaneous mutation in C57BL/Ka mice leads to the development of skin lesions at 5 to 6 weeks of age in homozygotes. Lesions occur in the dorsal and ventral skin, not in ears, footpads, or tail. Epidermal hyperplasia, hyper- and parakeratosis, and keratinocyte necrosis characterize the lesions. Granulocytes and macrophages infiltrate the dermis and epidermis, and there is an increase with age of the number of dermal mast cells. Mouth, esophagus, and forestomach develop similar lesions, and there is macrophage and neutrophil infiltration of liver, lungs, and joints. The long-acting corticosteroid triamcinolone causes regression of the lesions over a four week period; cyclosporin treatment had no effect (J:14539).
Molecular Note A single base pair deletion in the 3' end of exon 1 creates a shift in the open reading frame predicted to cause a premature stop codon at position 624. [MGI Ref ID J:121810]

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls

Colony Maintenance

Diet Information LabDiet® 5K52/5K67

Animal Health Reports

Room Number           FGB29

Research Applications

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

Dermatology Research
Skin and Hair Texture Defects

Immunology and Inflammation Research
Inflammation
Lymphoid Tissue Defects

References

Selected Reference(s)

Gijbels MJ; Zurcher C; Kraal G; Elliott GR; HogenEsch H; Schijff G ; Savelkoul HF ; Bruijnzeel PL. 1996. Pathogenesis of skin lesions in mice with chronic proliferative dermatitis (cpdm/cpdm). Am J Pathol 148(3):941-50. [PubMed: 8774148]  [MGI Ref ID J:31745]

HogenEsch H; Gijbels MJ; Offerman E; van Hooft J; van Bekkum DW; Zurcher C. 1993. A spontaneous mutation characterized by chronic proliferative dermatitis in C57BL mice. Am J Pathol 143(3):972-82. [PubMed: 8362989]  [MGI Ref ID J:14539]

HogenEsch H; Janke S; Boggess D; Sundberg JP. 1999. Absence of Peyer's patches and abnormal lymphoid architecture in chronic proliferative dermatitis (cpdm/cpdm) mice. J Immunol 162(7):3890-6. [PubMed: 10201907]  [MGI Ref ID J:53575]

Additional References

Price and Supply Information

Strain Name: C57BL/KaLawRij-Sharpincpdm/RijSunJ
Stock Number: 007599

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 Mouse Mutant Resource collection.
LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

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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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