Strain Name:

129S6/SvEvTac-Wastm1Sbs/J

Stock Number:

003292

Order this mouse

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

Type Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
 
Donating InvestigatorDr. Frederick W. Alt,   Children's Hospital

Description
WAS-deficient mice are viable and fertile. Mutant mice show normal lymphocyte development, serum immunoglobulin (Ig) levels and antibody responses. However, peripheral blood lymphocyte counts and platelet numbers are reduced in these mice. Development of chronic colitis is also observed. In vitro, WAS-deficient T cells show markedly impaired proliferative responses to anti-CD3e mediated stimulation. The Was gene is X-linked, so hemizygous males are WAS deficient.

Control Information

  Control
   002448 129S1/SvImJ (approximate)
   000691 129X1/SvJ (approximate)
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Wastm1Sbs allele
019458   B6.129S6-Wastm1Sbs/J
View Strains carrying   Wastm1Sbs     (1 strain)

Additional Web Information

New 129 Nomenclature Bulletin

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.
Iga Nephropathy, Susceptibility to, 1; IGAN1
Wiskott-Aldrich Syndrome; WAS
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Neutropenia, Severe Congenital, X-Linked; SCNX   (WAS)
Thrombocytopenia 1; THC1   (WAS)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Wastm1Sbs/Wastm1Sbs

        either: (involves: 129S6/SvEvTac) or (involves: 129S6/SvEvTac * C57BL/6)
  • tumorigenesis
  • *normal* tumorigenesis
    • young mutant mice develop neither hematopoeitic malignancies nor eczema   (MGI Ref ID J:48836)
  • digestive/alimentary phenotype
  • abnormal crypts of Lieberkuhn morphology
    • severely affected colons appear dilated with thickened walls   (MGI Ref ID J:48836)
    • the intestinal mucosa appears thickened with crypt hyperplasia and a mixed lymphocytic and neutrophilic infiltrate within the lamina propria   (MGI Ref ID J:48836)
    • crypts of Lieberkuhn abscesses
      • mutants with severe colitis exhibit crypt abscesses   (MGI Ref ID J:48836)
  • colitis
    • most mutants develop chronic colitis by 4 months of age   (MGI Ref ID J:48836)
    • large numbers of CD4+ and CD8+ T cells (but not B220+ B cells) are scattered throughout the lamina propria in affected mutants   (MGI Ref ID J:48836)
  • endocrine/exocrine gland phenotype
  • abnormal crypts of Lieberkuhn morphology
    • severely affected colons appear dilated with thickened walls   (MGI Ref ID J:48836)
    • the intestinal mucosa appears thickened with crypt hyperplasia and a mixed lymphocytic and neutrophilic infiltrate within the lamina propria   (MGI Ref ID J:48836)
    • crypts of Lieberkuhn abscesses
      • mutants with severe colitis exhibit crypt abscesses   (MGI Ref ID J:48836)
  • hematopoietic system phenotype
  • decreased T cell proliferation
    • in vitro, mutant T cells show defective proliferative responses and antigen receptor cap formation in reponse to anti-CD3epsilon mediated stimulation   (MGI Ref ID J:48836)
  • decreased lymphocyte cell number
    • mutants show a significant reduction in the number of peripheral blood lymphocytes, despite normal lymphocyte numbers in the spleen and lymph nodes   (MGI Ref ID J:48836)
    • young mutant mice (<10 weeks) exhibit comparable decreases in B and T cell numbers in peripheral blood, with no significant changes in blood B to T cell ratios   (MGI Ref ID J:48836)
    • decreased B cell number   (MGI Ref ID J:48836)
    • decreased T cell number   (MGI Ref ID J:48836)
  • decreased platelet cell number
    • mutants display modestly reduced numbers of platelets relative to wild-type mice   (MGI Ref ID J:48836)
    • notably, decreased platelet count is not associated with reduced platelet size or bleeding   (MGI Ref ID J:48836)
  • increased neutrophil cell number
    • in mutant mice, lymphopenia is associated with slightly increased numbers of neutrophils but normal numbers of red blood cells   (MGI Ref ID J:48836)
  • immune system phenotype
  • *normal* immune system phenotype
    • mutants are viable, fertile and of normal weight, and exhibit normal lymphocyte development, serum immunoglobulin (Ig) levels and antibody responses relative to wild-type mice   (MGI Ref ID J:48836)
    • colitis
      • most mutants develop chronic colitis by 4 months of age   (MGI Ref ID J:48836)
      • large numbers of CD4+ and CD8+ T cells (but not B220+ B cells) are scattered throughout the lamina propria in affected mutants   (MGI Ref ID J:48836)
    • decreased T cell proliferation
      • in vitro, mutant T cells show defective proliferative responses and antigen receptor cap formation in reponse to anti-CD3epsilon mediated stimulation   (MGI Ref ID J:48836)
    • decreased lymphocyte cell number
      • mutants show a significant reduction in the number of peripheral blood lymphocytes, despite normal lymphocyte numbers in the spleen and lymph nodes   (MGI Ref ID J:48836)
      • young mutant mice (<10 weeks) exhibit comparable decreases in B and T cell numbers in peripheral blood, with no significant changes in blood B to T cell ratios   (MGI Ref ID J:48836)
      • decreased B cell number   (MGI Ref ID J:48836)
      • decreased T cell number   (MGI Ref ID J:48836)
    • increased neutrophil cell number
      • in mutant mice, lymphopenia is associated with slightly increased numbers of neutrophils but normal numbers of red blood cells   (MGI Ref ID J:48836)

Wastm1Sbs/Wastm1Sbs

        involves: 129S/SvEv
  • cellular phenotype
  • abnormal cell migration
    • in vitro, bone marrow cells from mutant mice show a 2-fold reduction in SDF-1-induced chemotaxis relative to wild-type cells   (MGI Ref ID J:84888)
  • abnormal osteoclast differentiation
    • when cultured on glass coverslips, 67.9% of bone marrow osteoclasts from mutant mice are devoid of podosomes, assembling actin plaques that colocalize with vinculin   (MGI Ref ID J:90542)
    • on glass coverslips, 30.3% also assemble abnormal actin rings that are depleted of actin and vinculin and contain few podosome-like structures in the ring   (MGI Ref ID J:90542)
    • when cultured on bone slices, 52.7% of mutant osteoclasts are completely devoid of podosomes and fail to form actin rings at sealing zones; 45.8% display only abnormal actin-rich plaques that are undetectable in wild-type cells   (MGI Ref ID J:90542)
    • mutant osteoclasts exhibit a 7.9%- and a 1.8%-fold increase in the area of spreading on glass and bone, respectively, relative to wild-type   (MGI Ref ID J:90542)
    • increased area of spreading is associated with a 5.7%- and a 1.7%-fold increase in the number of nuclei per cell on glass and bone, respectively   (MGI Ref ID J:90542)
    • mutant osteoclasts are overall larger than wild-type but, in contrast to wild-type, do not undergo any further increase when plated on bone   (MGI Ref ID J:90542)
  • hematopoietic system phenotype
  • abnormal bone marrow cell morphology/development
    • serial transplantation and competitive reconstitution expts show that marrow cells, including hematopoietic progenitors and stem cells (HSCs), from heterozgous carriers exhibit decreased migration and homing capacities relative to wild-type cells   (MGI Ref ID J:84888)
    • despite a normal marrow and spleen hematopoiesis, HSCs from mutant mice display a defect in their ability to colonize hematopoietic tissues associated with a defect in adhesion to collagen I   (MGI Ref ID J:84888)
    • HSCs expressing a normal gene appear to have a selective advantage over deficient cells in their capacity to migrate and home to the bone marrow   (MGI Ref ID J:84888)
    • authors propose that nonrandom X inactivation in heterozygous females is the consequence of a defect in the homing capacities of HSCs during fetal life   (MGI Ref ID J:84888)
  • abnormal mast cell morphology
    • mutant BMMC adhere to IgE-coated plates but are impaired in their ability to spread and form ruffles   (MGI Ref ID J:86835)
  • abnormal mast cell physiology
    • after 4 weeks of culture in IL3, bone marrow-derived mast cells (BMMC) from mutant mice show similar kinetics of cell growth and differentiation as wild-type BMMC   (MGI Ref ID J:86835)
    • notably, the numbers of tissue mast cells in ears of mutant mice are comparable to those of wild-type   (MGI Ref ID J:86835)
    • in vivo, mutant BMMC display diminished IgE-mediated mast cell degranulation with a significantly reduced rise in plasma histamine levels relative to wild-type   (MGI Ref ID J:86835)
    • in vitro, mutant BMMC display reduced IgE-mediated mast cell degranulation with a significant reduction in beta-hexosaminidase release relative to wild-type after IgE cross-linking   (MGI Ref ID J:86835)
    • in addition, mutant BMMC show reduced Ca2+ mobilization and deficient JNK activation after IgE cross-linking   (MGI Ref ID J:86835)
  • abnormal osteoclast differentiation
    • when cultured on glass coverslips, 67.9% of bone marrow osteoclasts from mutant mice are devoid of podosomes, assembling actin plaques that colocalize with vinculin   (MGI Ref ID J:90542)
    • on glass coverslips, 30.3% also assemble abnormal actin rings that are depleted of actin and vinculin and contain few podosome-like structures in the ring   (MGI Ref ID J:90542)
    • when cultured on bone slices, 52.7% of mutant osteoclasts are completely devoid of podosomes and fail to form actin rings at sealing zones; 45.8% display only abnormal actin-rich plaques that are undetectable in wild-type cells   (MGI Ref ID J:90542)
    • mutant osteoclasts exhibit a 7.9%- and a 1.8%-fold increase in the area of spreading on glass and bone, respectively, relative to wild-type   (MGI Ref ID J:90542)
    • increased area of spreading is associated with a 5.7%- and a 1.7%-fold increase in the number of nuclei per cell on glass and bone, respectively   (MGI Ref ID J:90542)
    • mutant osteoclasts are overall larger than wild-type but, in contrast to wild-type, do not undergo any further increase when plated on bone   (MGI Ref ID J:90542)
  • abnormal osteoclast physiology
    • following ovariectomy, mutants exhibit a non-significant 16.4% of bone loss relative to wild-type (38.6%)   (MGI Ref ID J:90542)
    • despite low levels of bone loss, ovariectomized mutants show a 2.75-fold increase in total eroded surface, as well as a 1.5- and a 1.7-fold increase in the % of bone surface covered by osteoclasts and the total number of osteoclasts, respectively; no such increases are detected in wild-type   (MGI Ref ID J:90542)
    • consistent with impaired bone resorption, mutant osteoclasts tend to form small excavations on bone slices in vitro   (MGI Ref ID J:90542)
  • immune system phenotype
  • abnormal cytokine secretion
    • mutant BMMC secrete significantly less IL6 and TNF than wild-type BMMC at 6, 24 and 48 h after IgE cross-linking   (MGI Ref ID J:86835)
  • abnormal mast cell morphology
    • mutant BMMC adhere to IgE-coated plates but are impaired in their ability to spread and form ruffles   (MGI Ref ID J:86835)
  • abnormal mast cell physiology
    • after 4 weeks of culture in IL3, bone marrow-derived mast cells (BMMC) from mutant mice show similar kinetics of cell growth and differentiation as wild-type BMMC   (MGI Ref ID J:86835)
    • notably, the numbers of tissue mast cells in ears of mutant mice are comparable to those of wild-type   (MGI Ref ID J:86835)
    • in vivo, mutant BMMC display diminished IgE-mediated mast cell degranulation with a significantly reduced rise in plasma histamine levels relative to wild-type   (MGI Ref ID J:86835)
    • in vitro, mutant BMMC display reduced IgE-mediated mast cell degranulation with a significant reduction in beta-hexosaminidase release relative to wild-type after IgE cross-linking   (MGI Ref ID J:86835)
    • in addition, mutant BMMC show reduced Ca2+ mobilization and deficient JNK activation after IgE cross-linking   (MGI Ref ID J:86835)
  • abnormal osteoclast differentiation
    • when cultured on glass coverslips, 67.9% of bone marrow osteoclasts from mutant mice are devoid of podosomes, assembling actin plaques that colocalize with vinculin   (MGI Ref ID J:90542)
    • on glass coverslips, 30.3% also assemble abnormal actin rings that are depleted of actin and vinculin and contain few podosome-like structures in the ring   (MGI Ref ID J:90542)
    • when cultured on bone slices, 52.7% of mutant osteoclasts are completely devoid of podosomes and fail to form actin rings at sealing zones; 45.8% display only abnormal actin-rich plaques that are undetectable in wild-type cells   (MGI Ref ID J:90542)
    • mutant osteoclasts exhibit a 7.9%- and a 1.8%-fold increase in the area of spreading on glass and bone, respectively, relative to wild-type   (MGI Ref ID J:90542)
    • increased area of spreading is associated with a 5.7%- and a 1.7%-fold increase in the number of nuclei per cell on glass and bone, respectively   (MGI Ref ID J:90542)
    • mutant osteoclasts are overall larger than wild-type but, in contrast to wild-type, do not undergo any further increase when plated on bone   (MGI Ref ID J:90542)
  • abnormal osteoclast physiology
    • following ovariectomy, mutants exhibit a non-significant 16.4% of bone loss relative to wild-type (38.6%)   (MGI Ref ID J:90542)
    • despite low levels of bone loss, ovariectomized mutants show a 2.75-fold increase in total eroded surface, as well as a 1.5- and a 1.7-fold increase in the % of bone surface covered by osteoclasts and the total number of osteoclasts, respectively; no such increases are detected in wild-type   (MGI Ref ID J:90542)
    • consistent with impaired bone resorption, mutant osteoclasts tend to form small excavations on bone slices in vitro   (MGI Ref ID J:90542)
  • skeleton phenotype
  • abnormal osteoclast differentiation
    • when cultured on glass coverslips, 67.9% of bone marrow osteoclasts from mutant mice are devoid of podosomes, assembling actin plaques that colocalize with vinculin   (MGI Ref ID J:90542)
    • on glass coverslips, 30.3% also assemble abnormal actin rings that are depleted of actin and vinculin and contain few podosome-like structures in the ring   (MGI Ref ID J:90542)
    • when cultured on bone slices, 52.7% of mutant osteoclasts are completely devoid of podosomes and fail to form actin rings at sealing zones; 45.8% display only abnormal actin-rich plaques that are undetectable in wild-type cells   (MGI Ref ID J:90542)
    • mutant osteoclasts exhibit a 7.9%- and a 1.8%-fold increase in the area of spreading on glass and bone, respectively, relative to wild-type   (MGI Ref ID J:90542)
    • increased area of spreading is associated with a 5.7%- and a 1.7%-fold increase in the number of nuclei per cell on glass and bone, respectively   (MGI Ref ID J:90542)
    • mutant osteoclasts are overall larger than wild-type but, in contrast to wild-type, do not undergo any further increase when plated on bone   (MGI Ref ID J:90542)
  • abnormal osteoclast physiology
    • following ovariectomy, mutants exhibit a non-significant 16.4% of bone loss relative to wild-type (38.6%)   (MGI Ref ID J:90542)
    • despite low levels of bone loss, ovariectomized mutants show a 2.75-fold increase in total eroded surface, as well as a 1.5- and a 1.7-fold increase in the % of bone surface covered by osteoclasts and the total number of osteoclasts, respectively; no such increases are detected in wild-type   (MGI Ref ID J:90542)
    • consistent with impaired bone resorption, mutant osteoclasts tend to form small excavations on bone slices in vitro   (MGI Ref ID J:90542)

Wastm1Sbs/Wastm1Sbs

        involves: 129S6/SvEvTac
  • immune system phenotype
  • abnormal dendritic cell morphology
    • bone marrow-derived dendritic cell podosome turnover is increased compared to in wild-type mice   (MGI Ref ID J:153247)
  • abnormal dendritic cell physiology
    • chemotaxis velocity bone marrow-derived dendritic cells in response to CCL3 is decreased compared to similarly treated wild-type cells   (MGI Ref ID J:153247)
    • migration of dendritic cells is impaired compared to wild-type cells   (MGI Ref ID J:153247)
    • dendritic cells fail to exhibit an increase in phagocytosis of latex beads unlike similarly treated wild-type cells   (MGI Ref ID J:153247)
  • abnormal lymphocyte morphology
    • the proportion of mature marginal zone to marginal zone precursors and T2 cells is decreased compared to in wild-type mice   (MGI Ref ID J:153247)
    • abnormal double-negative T cell morphology
      • the ratio of double negative 3 (DN3) to DN4 is skewed (1.49+/-0.90 compared to 0.74+/-0.45 in wild-type mice)   (MGI Ref ID J:125309)
    • abnormal double-positive T cell morphology
      • the percent of double positive (DP) CD69high cells is greater than in wild-type   (MGI Ref ID J:125309)
    • abnormal thymocyte activation
      • unlike wild-type thymocytes, single positive (SP) thymocytes fail to proliferate after CD3 stimulation   (MGI Ref ID J:125309)
      • spreading of TCRhigh CD4 or CD8 SP thymocytes on a surface covered with antibodies to CD3 and CD28 is reduced compared to wild-type cells   (MGI Ref ID J:125309)
      • migratory responses to CCL19 or CXCL12 are reduced compared to in wild-type cells   (MGI Ref ID J:125309)
    • decreased NK cell number
      • in the spleen   (MGI Ref ID J:153247)
    • decreased T cell number
      • mice have fewer CD4+ CD3+ and CD8+ CD3+ T cells than in wild-type mice   (MGI Ref ID J:153247)
      • fewer CD3+ T cells, especially CD8+CD3+ and CD4+CD8+Cd3+ T cells, are observed in the spleen, lymph nodes, and thymus than in wild-type mice   (MGI Ref ID J:153247)
    • decreased T cell proliferation   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
  • increased anti-double stranded DNA antibody level
    • at a low titer   (MGI Ref ID J:153247)
  • increased leukocyte cell number   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
    • increased monocyte cell number   (MGI Ref ID J:153247)
  • increased splenocyte number   (MGI Ref ID J:153247)
  • spleen hyperplasia   (MGI Ref ID J:153247)
  • hematopoietic system phenotype
  • abnormal dendritic cell morphology
    • bone marrow-derived dendritic cell podosome turnover is increased compared to in wild-type mice   (MGI Ref ID J:153247)
  • abnormal lymphocyte morphology
    • the proportion of mature marginal zone to marginal zone precursors and T2 cells is decreased compared to in wild-type mice   (MGI Ref ID J:153247)
    • abnormal double-negative T cell morphology
      • the ratio of double negative 3 (DN3) to DN4 is skewed (1.49+/-0.90 compared to 0.74+/-0.45 in wild-type mice)   (MGI Ref ID J:125309)
    • abnormal double-positive T cell morphology
      • the percent of double positive (DP) CD69high cells is greater than in wild-type   (MGI Ref ID J:125309)
    • abnormal thymocyte activation
      • unlike wild-type thymocytes, single positive (SP) thymocytes fail to proliferate after CD3 stimulation   (MGI Ref ID J:125309)
      • spreading of TCRhigh CD4 or CD8 SP thymocytes on a surface covered with antibodies to CD3 and CD28 is reduced compared to wild-type cells   (MGI Ref ID J:125309)
      • migratory responses to CCL19 or CXCL12 are reduced compared to in wild-type cells   (MGI Ref ID J:125309)
    • decreased NK cell number
      • in the spleen   (MGI Ref ID J:153247)
    • decreased T cell number
      • mice have fewer CD4+ CD3+ and CD8+ CD3+ T cells than in wild-type mice   (MGI Ref ID J:153247)
      • fewer CD3+ T cells, especially CD8+CD3+ and CD4+CD8+Cd3+ T cells, are observed in the spleen, lymph nodes, and thymus than in wild-type mice   (MGI Ref ID J:153247)
    • decreased T cell proliferation   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
  • decreased platelet cell number   (MGI Ref ID J:153247)
  • increased leukocyte cell number   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
    • increased monocyte cell number   (MGI Ref ID J:153247)
  • increased splenocyte number   (MGI Ref ID J:153247)
  • spleen hyperplasia   (MGI Ref ID J:153247)
  • endocrine/exocrine gland phenotype
  • abnormal thymocyte activation
    • unlike wild-type thymocytes, single positive (SP) thymocytes fail to proliferate after CD3 stimulation   (MGI Ref ID J:125309)
    • spreading of TCRhigh CD4 or CD8 SP thymocytes on a surface covered with antibodies to CD3 and CD28 is reduced compared to wild-type cells   (MGI Ref ID J:125309)
    • migratory responses to CCL19 or CXCL12 are reduced compared to in wild-type cells   (MGI Ref ID J:125309)

Wastm1Sbs/Wastm1Sbs

        129S6/SvEvTac-Wastm1Sbs/J
  • homeostasis/metabolism phenotype
  • increased urine protein level
    • mutants older than 6 months of age exhibit proteinuria   (MGI Ref ID J:180407)
  • immune system phenotype
  • glomerulonephritis
    • mutants develop proliferative glomerulonephritis similar to human IgA nephropathy   (MGI Ref ID J:180407)
  • increased IgA level
    • elevation in serum IgA, both in young and old mutants   (MGI Ref ID J:180407)
    • B cells exhibit increased IgA production after stimulation with LPS, TGF-beta1, IL-4, and IL-5   (MGI Ref ID J:180407)
    • reduced ratio of sialylated and galactosylated IgA in the serum, indicating that glycosylation of IgA is abnormal in mutants   (MGI Ref ID J:180407)
    • mutants exhibit higher titers of circulating IgA-containing complexes   (MGI Ref ID J:180407)
    • mutants exhibit increased glomerular IgA deposition in the kidneys   (MGI Ref ID J:180407)
  • increased IgM level
    • mutants exhibit increased glomerular IgM and C3 complement deposition in the kidneys   (MGI Ref ID J:180407)
  • renal/urinary system phenotype
  • abnormal kidney morphology
    • mutants exhibit renal injury with increasing severity with advancing age   (MGI Ref ID J:180407)
    • abnormal glomerular mesangium morphology
      • mutants older than 6 months of age exhibit hump-like mesangial and paramesangial deposits   (MGI Ref ID J:180407)
      • expanded mesangial matrix
        • mutants older than 6 months of age, exhibit increased matrix deposition with or without endocapillary proliferation   (MGI Ref ID J:180407)
      • mesangial cell hyperplasia
        • mutants older than 6 months of age, exhibit increased mesangial cellularity with or without endocapillary proliferation   (MGI Ref ID J:180407)
    • glomerulonephritis
      • mutants develop proliferative glomerulonephritis similar to human IgA nephropathy   (MGI Ref ID J:180407)
  • increased urine protein level
    • mutants older than 6 months of age exhibit proteinuria   (MGI Ref ID J:180407)
  • hematopoietic system phenotype
  • increased IgA level
    • elevation in serum IgA, both in young and old mutants   (MGI Ref ID J:180407)
    • B cells exhibit increased IgA production after stimulation with LPS, TGF-beta1, IL-4, and IL-5   (MGI Ref ID J:180407)
    • reduced ratio of sialylated and galactosylated IgA in the serum, indicating that glycosylation of IgA is abnormal in mutants   (MGI Ref ID J:180407)
    • mutants exhibit higher titers of circulating IgA-containing complexes   (MGI Ref ID J:180407)
    • mutants exhibit increased glomerular IgA deposition in the kidneys   (MGI Ref ID J:180407)
  • increased IgM level
    • mutants exhibit increased glomerular IgM and C3 complement deposition in the kidneys   (MGI Ref ID J:180407)

Wastm1Sbs/Y

        involves: 129S6/SvEvTac
  • immune system phenotype
  • abnormal dendritic cell morphology
    • bone marrow-derived dendritic cell podosome turnover is increased compared to in wild-type mice   (MGI Ref ID J:153247)
  • abnormal dendritic cell physiology
    • chemotaxis velocity bone marrow-derived dendritic cells in response to CCL3 is decreased compared to similarly treated wild-type cells   (MGI Ref ID J:153247)
    • migration of dendritic cells is impaired compared to wild-type cells   (MGI Ref ID J:153247)
    • dendritic cells fail to exhibit an increase in phagocytosis of latex beads unlike similarly treated wild-type cells   (MGI Ref ID J:153247)
  • abnormal lymphocyte morphology
    • the proportion of mature marginal zone to marginal zone precursors and T2 cells is decreased compared to in wild-type mice   (MGI Ref ID J:153247)
    • decreased NK cell number
      • in the spleen   (MGI Ref ID J:153247)
    • decreased T cell number
      • mice have fewer CD4+ CD3+ and CD8+ CD3+ T cells than in wild-type mice   (MGI Ref ID J:153247)
      • fewer CD3+ T cells, especially CD8+CD3+ and CD4+CD8+Cd3+ T cells, are observed in the spleen, lymph nodes, and thymus than in wild-type mice   (MGI Ref ID J:153247)
    • decreased T cell proliferation   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
  • increased anti-double stranded DNA antibody level
    • at a low titer   (MGI Ref ID J:153247)
  • increased leukocyte cell number   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
    • increased monocyte cell number   (MGI Ref ID J:153247)
  • increased splenocyte number   (MGI Ref ID J:153247)
  • spleen hyperplasia   (MGI Ref ID J:153247)
  • hematopoietic system phenotype
  • abnormal dendritic cell morphology
    • bone marrow-derived dendritic cell podosome turnover is increased compared to in wild-type mice   (MGI Ref ID J:153247)
  • abnormal lymphocyte morphology
    • the proportion of mature marginal zone to marginal zone precursors and T2 cells is decreased compared to in wild-type mice   (MGI Ref ID J:153247)
    • decreased NK cell number
      • in the spleen   (MGI Ref ID J:153247)
    • decreased T cell number
      • mice have fewer CD4+ CD3+ and CD8+ CD3+ T cells than in wild-type mice   (MGI Ref ID J:153247)
      • fewer CD3+ T cells, especially CD8+CD3+ and CD4+CD8+Cd3+ T cells, are observed in the spleen, lymph nodes, and thymus than in wild-type mice   (MGI Ref ID J:153247)
    • decreased T cell proliferation   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
  • decreased platelet cell number   (MGI Ref ID J:153247)
  • increased leukocyte cell number   (MGI Ref ID J:153247)
    • increased B cell number
      • mice exhibit an increase in B220+ B cells in the spleen compared with wild-type mice   (MGI Ref ID J:153247)
      • increased follicular B cell number   (MGI Ref ID J:153247)
    • increased monocyte cell number   (MGI Ref ID J:153247)
  • increased splenocyte number   (MGI Ref ID J:153247)
  • spleen hyperplasia   (MGI Ref ID J:153247)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Wastm1Sbs/Wastm1Sbs

        involves: C57BL/6 * CBA
  • immune system phenotype
  • abnormal B cell physiology
    • mutant B cells display notable defects in polarization, spreading, and microvilli formation in response to IL-4- and CD40-dependent stimuli relative to similarly-induced wild-type B cells; homotypic aggregation is only mildly impaired   (MGI Ref ID J:95909)
    • activated mutant B lymphocytes exhibit an abnormally smooth surface and express shorter microvilli that are less dense in cell contacts than in activated wild-type B cells   (MGI Ref ID J:95909)
  • hematopoietic system phenotype
  • abnormal B cell physiology
    • mutant B cells display notable defects in polarization, spreading, and microvilli formation in response to IL-4- and CD40-dependent stimuli relative to similarly-induced wild-type B cells; homotypic aggregation is only mildly impaired   (MGI Ref ID J:95909)
    • activated mutant B lymphocytes exhibit an abnormally smooth surface and express shorter microvilli that are less dense in cell contacts than in activated wild-type B cells   (MGI Ref ID J:95909)

Wastm1Sbs/Wastm1Sbs

        involves: BALB/c
  • immune system phenotype
  • *normal* immune system phenotype
    • in vitro, activated mutant B cells and DCs process and present soluble ovalbumin (whole OVA) or OVA 323-339 petides efficiently, with normal proliferative and cytokine responses in TCR tg CD4+ T cells   (MGI Ref ID J:84131)
    • notably, LPS-activated B cells, lacking microvilli, and (LPS+IL-4)-activated B cells, possessing many and long microvilli, exhibit a similar antigen processing and presenting capacity   (MGI Ref ID J:84131)
    • in vivo, immunization of mutant mice with OVA elicits proliferation of transferred, fluorescent-labelled, CD4+ T cells, confirming normal processing of soluble antigen and presentation on MHC class II molecules by APCs   (MGI Ref ID J:84131)
    • abnormal dendritic cell antigen presentation
      • DCs from mutant mice show impaired processing and presentation of particulate antigen (bacterial-expressed OVA)   (MGI Ref ID J:84131)
    • decreased T cell proliferation
      • mutant DCs present bacterial-derived OVA peptides but the T cell response is less than 50% of wild-type DCs, suggesting impaired processing of particulate antigen in DCs   (MGI Ref ID J:84131)
  • hematopoietic system phenotype
  • decreased T cell proliferation
    • mutant DCs present bacterial-derived OVA peptides but the T cell response is less than 50% of wild-type DCs, suggesting impaired processing of particulate antigen in DCs   (MGI Ref ID J:84131)

Wastm1Sbs/Wastm1Sbs

        involves: 129S6/SvEvTac * C57BL/6
  • immune system phenotype
  • abnormal B cell physiology
    • virtual abrogation of IgM and IgG pneumococcus polysaccharide specific Ab responses   (MGI Ref ID J:182528)
    • abrogation of the early response to immunization with UV inactivated vesicular stomatitis virus   (MGI Ref ID J:182528)
    • increased IgA level   (MGI Ref ID J:182528)
    • increased IgE level   (MGI Ref ID J:182528)
    • increased IgM level
      • elevated total serum levels and spontaneous production of IgM Abs specific for trinitrophenyl and phosphocholine   (MGI Ref ID J:182528)
  • decreased mature B cell number
    • decrease in the percentage and absolute number of bone marrow B220 hi mature recirculating B cells   (MGI Ref ID J:182528)
    • decreased marginal zone B cell number
      • decrease in the percentage and absolute number of marginal zone B cells   (MGI Ref ID J:182528)
      • the ring of B220+ CD1d+ MZ B cells that normally surrounds MOMA-expressing metallophilic macrophages is almost absent   (MGI Ref ID J:182528)
  • increased autoantibody level   (MGI Ref ID J:182528)
    • increased anti-chromatin antibody level   (MGI Ref ID J:182528)
    • increased anti-double stranded DNA antibody level   (MGI Ref ID J:182528)
    • increased anti-single stranded DNA antibody level   (MGI Ref ID J:182528)
  • increased germinal center B cell number
    • in the spleen and lymph nodes   (MGI Ref ID J:182528)
  • increased plasma cell number
    • increase in the proportion of CD19+ CD138hi plasma cells   (MGI Ref ID J:182528)
  • renal/urinary system phenotype
  • abnormal renal corpuscle morphology
    • modest degree of glomerular damage   (MGI Ref ID J:182528)
  • hematopoietic system phenotype
  • abnormal B cell physiology
    • virtual abrogation of IgM and IgG pneumococcus polysaccharide specific Ab responses   (MGI Ref ID J:182528)
    • abrogation of the early response to immunization with UV inactivated vesicular stomatitis virus   (MGI Ref ID J:182528)
    • increased IgA level   (MGI Ref ID J:182528)
    • increased IgE level   (MGI Ref ID J:182528)
    • increased IgM level
      • elevated total serum levels and spontaneous production of IgM Abs specific for trinitrophenyl and phosphocholine   (MGI Ref ID J:182528)
  • abnormal megakaryocyte morphology
    • megakaryocytes fail to form podosomes when spread on collagen   (MGI Ref ID J:195436)
    • megakaryocytes exhibit reduced crossing protrussions compared with wild-type cells   (MGI Ref ID J:195436)
    • however, megakaryocytes spread normally on both collagen and fibrinogen   (MGI Ref ID J:195436)
    • decreased platelet cell number   (MGI Ref ID J:182528)
  • decreased mature B cell number
    • decrease in the percentage and absolute number of bone marrow B220 hi mature recirculating B cells   (MGI Ref ID J:182528)
    • decreased marginal zone B cell number
      • decrease in the percentage and absolute number of marginal zone B cells   (MGI Ref ID J:182528)
      • the ring of B220+ CD1d+ MZ B cells that normally surrounds MOMA-expressing metallophilic macrophages is almost absent   (MGI Ref ID J:182528)
  • increased germinal center B cell number
    • in the spleen and lymph nodes   (MGI Ref ID J:182528)
  • increased plasma cell number
    • increase in the proportion of CD19+ CD138hi plasma cells   (MGI Ref ID J:182528)

Wastm1Sbs/Y

        involves: 129S6/SvEvTac * C57BL/6
  • immune system phenotype
  • abnormal B cell physiology
    • virtual abrogation of IgM and IgG pneumococcus polysaccharide specific Ab responses   (MGI Ref ID J:182528)
    • abrogation of the early response to immunization with UV inactivated vesicular stomatitis virus   (MGI Ref ID J:182528)
    • increased IgA level   (MGI Ref ID J:182528)
    • increased IgE level   (MGI Ref ID J:182528)
    • increased IgM level
      • elevated total serum levels and spontaneous production of IgM Abs specific for trinitrophenyl and phosphocholine   (MGI Ref ID J:182528)
  • decreased mature B cell number
    • decrease in the percentage and absolute number of bone marrow B220 hi mature recirculating B cells   (MGI Ref ID J:182528)
    • decreased marginal zone B cell number
      • decrease in the percentage and absolute number of marginal zone B cells   (MGI Ref ID J:182528)
      • the ring of B220+ CD1d+ MZ B cells that normally surrounds MOMA-expressing metallophilic macrophages is almost absent   (MGI Ref ID J:182528)
  • increased autoantibody level   (MGI Ref ID J:182528)
    • increased anti-chromatin antibody level   (MGI Ref ID J:182528)
    • increased anti-double stranded DNA antibody level   (MGI Ref ID J:182528)
    • increased anti-single stranded DNA antibody level   (MGI Ref ID J:182528)
  • increased germinal center B cell number
    • in the spleen and lymph nodes   (MGI Ref ID J:182528)
  • increased plasma cell number
    • increase in the proportion of CD19+ CD138hi plasma cells   (MGI Ref ID J:182528)
  • renal/urinary system phenotype
  • abnormal renal corpuscle morphology
    • modest degree of glomerular damage   (MGI Ref ID J:182528)
  • hematopoietic system phenotype
  • abnormal B cell physiology
    • virtual abrogation of IgM and IgG pneumococcus polysaccharide specific Ab responses   (MGI Ref ID J:182528)
    • abrogation of the early response to immunization with UV inactivated vesicular stomatitis virus   (MGI Ref ID J:182528)
    • increased IgA level   (MGI Ref ID J:182528)
    • increased IgE level   (MGI Ref ID J:182528)
    • increased IgM level
      • elevated total serum levels and spontaneous production of IgM Abs specific for trinitrophenyl and phosphocholine   (MGI Ref ID J:182528)
  • decreased mature B cell number
    • decrease in the percentage and absolute number of bone marrow B220 hi mature recirculating B cells   (MGI Ref ID J:182528)
    • decreased marginal zone B cell number
      • decrease in the percentage and absolute number of marginal zone B cells   (MGI Ref ID J:182528)
      • the ring of B220+ CD1d+ MZ B cells that normally surrounds MOMA-expressing metallophilic macrophages is almost absent   (MGI Ref ID J:182528)
  • decreased platelet cell number   (MGI Ref ID J:182528)
  • increased germinal center B cell number
    • in the spleen and lymph nodes   (MGI Ref ID J:182528)
  • increased plasma cell number
    • increase in the proportion of CD19+ CD138hi plasma cells   (MGI Ref ID J:182528)
View Research Applications

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

Wastm1Sbs related

Hematological Research
Immunological Defects
Platelet Defects

Immunology, Inflammation and Autoimmunity Research
Immunodeficiency
Inflammation
      Inflammatory bowel disease
Intracellular Signaling Molecules
Lymphoid Tissue Defects
T Cell Receptor Signaling Defects

Internal/Organ Research
Gastrointestinal Defects
      colitis
Lymphoid Tissue Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Wastm1Sbs
Allele Name targeted mutation 1, Scott B Snapper
Allele Type Targeted (Null/Knockout)
Common Name(s) Was-; wasp-;
Mutation Made ByDr. Scott Snapper,   Massachusetts General Hospital
Strain of Origin129S6/SvEvTac
ES Cell Line NameTC1/TC-1
ES Cell Line Strain129S6/SvEvTac
Gene Symbol and Name Was, Wiskott-Aldrich syndrome homolog (human)
Chromosome X
Gene Common Name(s) IMD2; SCNX; THC; THC1; U42471; WASP; Wasp; Wiskott-Aldrich syndrome protein; expressed sequence U42471;
Molecular Note A neomycin cassette was inserted into exon 7, which encodes part of the GTPase-binding domain. The absence of transcript and protein produced from the targeted allele in male hemizygous mutant mice was confirmed by Northern blot and Western blot analyses. [MGI Ref ID J:48836]

Genotyping

Genotyping Information

Genotyping Protocols

Wastm1Sbs, Separated PCR
Wastm1Sbs, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Snapper SB; Rosen FS; Mizoguchi E; Cohen P; Khan W; Liu CH; Hagemann TL; Kwan SP; Ferrini R; Davidson L; Bhan AK; Alt FW. 1998. Wiskott-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation. Immunity 9(1):81-91. [PubMed: 9697838]  [MGI Ref ID J:48836]

Additional References

Cannon JL; Burkhardt JK. 2004. Differential roles for Wiskott-Aldrich syndrome protein in immune synapse formation and IL-2 production. J Immunol 173(3):1658-62. [PubMed: 15265894]  [MGI Ref ID J:92036]

Wastm1Sbs related

Adriani M; Aoki J; Horai R; Thornton AM; Konno A; Kirby M; Anderson SM; Siegel RM; Candotti F; Schwartzberg PL. 2007. Impaired in vitro regulatory T cell function associated with Wiskott-Aldrich syndrome. Clin Immunol 124(1):41-8. [PubMed: 17512803]  [MGI Ref ID J:123591]

Adriani M; Jones KA; Uchiyama T; Kirby MR; Silvin C; Anderson SM; Candotti F. 2011. Defective inhibition of B-cell proliferation by Wiskott-Aldrich syndrome protein-deficient regulatory T cells. Blood 117(24):6608-11. [PubMed: 21515824]  [MGI Ref ID J:174827]

Andreansky S; Liu H; Turner S; McCullers JA; Lang R; Rutschman R; Doherty PC; Murray PJ; Nienhuis AW; Strom TS. 2005. WASP(-) mice exhibit defective immune responses to influenza A virus, Streptococcus pneumoniae, and Mycobacterium bovis BCG. Exp Hematol 33(4):443-51. [PubMed: 15781335]  [MGI Ref ID J:97098]

Astrakhan A; Sather BD; Ryu BY; Khim S; Singh S; Humblet-Baron S; Ochs HD; Miao CH; Rawlings DJ. 2012. Ubiquitous high-level gene expression in hematopoietic lineages provides effective lentiviral gene therapy of murine Wiskott-Aldrich syndrome. Blood 119(19):4395-407. [PubMed: 22431569]  [MGI Ref ID J:185176]

Blundell MP; Bouma G; Metelo J; Worth A; Calle Y; Cowell LA; Westerberg LS; Moulding DA; Mirando S; Kinnon C; Cory GO; Jones GE; Snapper SB; Burns SO; Thrasher AJ. 2009. Phosphorylation of WASp is a key regulator of activity and stability in vivo. Proc Natl Acad Sci U S A 106(37):15738-43. [PubMed: 19805221]  [MGI Ref ID J:153247]

Bouma G; Burns S; Thrasher AJ. 2007. Impaired T-cell priming in vivo resulting from dysfunction of WASp-deficient dendritic cells. Blood 110(13):4278-84. [PubMed: 17673604]  [MGI Ref ID J:149117]

Bouma G; Mendoza-Naranjo A; Blundell MP; de Falco E; Parsley KL; Burns SO; Thrasher AJ. 2011. Cytoskeletal remodeling mediated by WASp in dendritic cells is necessary for normal immune synapse formation and T-cell priming. Blood 118(9):2492-501. [PubMed: 21690559]  [MGI Ref ID J:176916]

Calle Y; Jones GE; Jagger C; Fuller K; Blundell MP; Chow J; Chambers T; Thrasher AJ. 2004. WASp deficiency in mice results in failure to form osteoclast sealing zones and defects in bone resorption. Blood 103(9):3552-61. [PubMed: 14726392]  [MGI Ref ID J:90542]

Chen Y; Aardema J; Kale S; Whichard ZL; Awomolo A; Blanchard E; Chang B; Myers DR; Ju L; Tran R; Reece D; Christensen H; Boukour S; Debili N; Strom TS; Rawlings D; Vazquez FX; Voth GA; Zhu C; Kahr WH; Lam WA; Corey SJ. 2013. Loss of the F-BAR protein CIP4 reduces platelet production by impairing membrane-cytoskeleton remodeling. Blood 122(10):1695-706. [PubMed: 23881916]  [MGI Ref ID J:202363]

Chou HC; Anton IM; Holt MR; Curcio C; Lanzardo S; Worth A; Burns S; Thrasher AJ; Jones GE; Calle Y. 2006. WIP regulates the stability and localization of WASP to podosomes in migrating dendritic cells. Curr Biol 16(23):2337-44. [PubMed: 17141616]  [MGI Ref ID J:117929]

Cotta-de-Almeida V; Westerberg L; Maillard MH; Onaldi D; Wachtel H; Meelu P; Chung UI; Xavier R; Alt FW; Snapper SB. 2007. Wiskott Aldrich syndrome protein (WASP) and N-WASP are critical for T cell development. Proc Natl Acad Sci U S A 104(39):15424-9. [PubMed: 17878299]  [MGI Ref ID J:125309]

Dehring DA; Clarke F; Ricart BG; Huang Y; Gomez TS; Williamson EK; Hammer DA; Billadeau DD; Argon Y; Burkhardt JK. 2011. Hematopoietic lineage cell-specific protein 1 functions in concert with the Wiskott-Aldrich syndrome protein to promote podosome array organization and chemotaxis in dendritic cells. J Immunol 186(8):4805-18. [PubMed: 21398607]  [MGI Ref ID J:172457]

Falet H; Marchetti MP; Hoffmeister KM; Massaad MJ; Geha RS; Hartwig JH. 2009. Platelet-associated IgAs and impaired GPVI responses in platelets lacking WIP. Blood 114(21):4729-37. [PubMed: 19692704]  [MGI Ref ID J:155166]

Gallego MD; de la Fuente MA; Anton IM; Snapper S; Fuhlbrigge R; Geha RS. 2006. WIP and WASP play complementary roles in T cell homing and chemotaxis to SDF-1{alpha}. Int Immunol 18(2):221-32. [PubMed: 16141245]  [MGI Ref ID J:106360]

Guerra S; Aracil M; Conde R; Bernad A; Esteban M. 2005. Wiskott-Aldrich syndrome protein is needed for vaccinia virus pathogenesis. J Virol 79(4):2133-40. [PubMed: 15681416]  [MGI Ref ID J:96040]

Humblet-Baron S; Sather B; Anover S; Becker-Herman S; Kasprowicz DJ; Khim S; Nguyen T; Hudkins-Loya K; Alpers CE; Ziegler SF; Ochs H; Torgerson T; Campbell DJ; Rawlings DJ. 2007. Wiskott-Aldrich syndrome protein is required for regulatory T cell homeostasis. J Clin Invest 117(2):407-418. [PubMed: 17218989]  [MGI Ref ID J:117933]

Isaac BM; Ishihara D; Nusblat LM; Gevrey JC; Dovas A; Condeelis J; Cox D. 2010. N-WASP has the ability to compensate for the loss of WASP in macrophage podosome formation and chemotaxis. Exp Cell Res 316(20):3406-16. [PubMed: 20599953]  [MGI Ref ID J:167791]

Ishihara D; Dovas A; Park H; Isaac BM; Cox D. 2012. The chemotactic defect in wiskott-Aldrich syndrome macrophages is due to the reduced persistence of directional protrusions. PLoS One 7(1):e30033. [PubMed: 22279563]  [MGI Ref ID J:184235]

Kim H; Falet H; Hoffmeister KM; Hartwig JH. 2013. Wiskott-Aldrich syndrome protein (WASp) controls the delivery of platelet transforming growth factor-beta1. J Biol Chem 288(48):34352-63. [PubMed: 24133214]  [MGI Ref ID J:204969]

Krawczyk C; Bachmaier K; Sasaki T; Jones GR; Snapper BS; Bouchard D; Kozieradzki I; Ohashi SP; Alt WF; Penninger MJ. 2000. Cbl-b is a negative regulator of receptor clustering and raft aggregation in T cells. Immunity 13(4):463-73. [PubMed: 11070165]  [MGI Ref ID J:79550]

Krawczyk C; Oliveira-dos-Santos A; Sasaki T; Griffiths E; Ohashi PS; Snapper S; Alt F; Penninger JM. 2002. Vav1 controls integrin clustering and MHC/peptide-specific cell adhesion to antigen-presenting cells. Immunity 16(3):331-43. [PubMed: 11911819]  [MGI Ref ID J:111206]

Kumar S; Xu J; Perkins C; Guo F; Snapper S; Finkelman FD; Zheng Y; Filippi MD. 2012. Cdc42 regulates neutrophil migration via crosstalk between WASp, CD11b, and microtubules. Blood 120(17):3563-74. [PubMed: 22932798]  [MGI Ref ID J:192929]

Lacout C; Haddad E; Sabri S; Svinarchouk F; Garcon L; Capron C; Foudi A; Mzali R; Snapper SB; Louache F; Vainchenker W; Dumenil D. 2003. A defect in hematopoietic stem cell migration explains the nonrandom X-chromosome inactivation in carriers of Wiskott-Aldrich syndrome. Blood 102(4):1282-9. [PubMed: 12730112]  [MGI Ref ID J:84888]

Le Bras S; Massaad M; Koduru S; Kumar L; Oyoshi MK; Hartwig J; Geha RS. 2009. WIP is critical for T cell responsiveness to IL-2. Proc Natl Acad Sci U S A 106(18):7519-24. [PubMed: 19359486]  [MGI Ref ID J:148334]

Liu C; Bai X; Wu J; Sharma S; Upadhyaya A; Dahlberg CI; Westerberg LS; Snapper SB; Zhao X; Song W. 2013. N-wasp is essential for the negative regulation of B cell receptor signaling. PLoS Biol 11(11):e1001704. [PubMed: 24223520]  [MGI Ref ID J:205106]

Liu C; Miller H; Hui KL; Grooman B; Bolland S; Upadhyaya A; Song W. 2011. A balance of Bruton's tyrosine kinase and SHIP activation regulates B cell receptor cluster formation by controlling actin remodeling. J Immunol 187(1):230-9. [PubMed: 21622861]  [MGI Ref ID J:175938]

Maillard MH; Cotta-de-Almeida V; Takeshima F; Nguyen DD; Michetti P; Nagler C; Bhan AK; Snapper SB. 2007. The Wiskott-Aldrich syndrome protein is required for the function of CD4(+)CD25(+)Foxp3(+) regulatory T cells. J Exp Med 204(2):381-91. [PubMed: 17296786]  [MGI Ref ID J:125368]

Mani M; Venkatasubrahmanyam S; Sanyal M; Levy S; Butte A; Weinberg K; Jahn T. 2009. Wiskott-Aldrich syndrome protein is an effector of Kit signaling. Blood 114(14):2900-8. [PubMed: 19643989]  [MGI Ref ID J:153286]

Marathe BM; Prislovsky A; Astrakhan A; Rawlings DJ; Wan JY; Strom TS. 2009. Antiplatelet antibodies in WASP(-) mice correlate with evidence of increased in vivo platelet consumption. Exp Hematol 37(11):1353-63. [PubMed: 19733207]  [MGI Ref ID J:154463]

Meyer-Bahlburg A; Becker-Herman S; Humblet-Baron S; Khim S; Weber M; Bouma G; Thrasher AJ; Batista FD; Rawlings DJ. 2008. Wiskott-Aldrich syndrome protein deficiency in B cells results in impaired peripheral homeostasis. Blood 112(10):4158-69. [PubMed: 18687984]  [MGI Ref ID J:142148]

Nguyen DD; Maillard MH; Cotta-de-Almeida V; Mizoguchi E; Klein C; Fuss I; Nagler C; Mizoguchi A; Bhan AK; Snapper SB. 2007. Lymphocyte-dependent and Th2 cytokine-associated colitis in mice deficient in Wiskott-Aldrich syndrome protein. Gastroenterology 133(4):1188-97. [PubMed: 17764675]  [MGI Ref ID J:128391]

Nikolov NP; Shimizu M; Cleland S; Bailey D; Aoki J; Strom T; Schwartzberg PL; Candotti F; Siegel RM. 2010. Systemic autoimmunity and defective Fas ligand secretion in the absence of the Wiskott-Aldrich syndrome protein. Blood 116(5):740-7. [PubMed: 20457871]  [MGI Ref ID J:163499]

Park H; Dovas A; Hanna S; Lastrucci C; Cougoule C; Guiet R; Maridonneau-Parini I; Cox D. 2014. Tyrosine phosphorylation of Wiskott-Aldrich syndrome protein (WASP) by Hck regulates macrophage function. J Biol Chem 289(11):7897-906. [PubMed: 24482227]  [MGI Ref ID J:210678]

Pivniouk VI; Snapper SB; Kettner A; Alenius H; Laouini D; Falet H; Hartwig J; Alt FW; Geha RS. 2003. Impaired signaling via the high-affinity IgE receptor in Wiskott-Aldrich syndrome protein-deficient mast cells. Int Immunol 15(12):1431-40. [PubMed: 14645152]  [MGI Ref ID J:86835]

Prete F; Catucci M; Labrada M; Gobessi S; Castiello MC; Bonomi E; Aiuti A; Vermi W; Cancrini C; Metin A; Hambleton S; Bredius R; Notarangelo LD; van der Burg M; Kalinke U; Villa A; Benvenuti F. 2013. Wiskott-Aldrich syndrome protein-mediated actin dynamics control type-I interferon production in plasmacytoid dendritic cells. J Exp Med 210(2):355-74. [PubMed: 23337808]  [MGI Ref ID J:196418]

Prislovsky A; Marathe B; Hosni A; Bolen AL; Nimmerjahn F; Jackson CW; Weiman D; Strom TS. 2008. Rapid platelet turnover in WASP(-) mice correlates with increased ex vivo phagocytosis of opsonized WASP(-) platelets. Exp Hematol 36(5):609-23. [PubMed: 18346836]  [MGI Ref ID J:136095]

Prislovsky A; Strom TS. 2013. Increased uptake by splenic red pulp macrophages contributes to rapid platelet turnover in WASP(-) mice. Exp Hematol 41(9):789-98. [PubMed: 23727585]  [MGI Ref ID J:214036]

Pulecio J; Tagliani E; Scholer A; Prete F; Fetler L; Burrone OR; Benvenuti F. 2008. Expression of Wiskott-Aldrich syndrome protein in dendritic cells regulates synapse formation and activation of naive CD8+ T cells. J Immunol 181(2):1135-42. [PubMed: 18606666]  [MGI Ref ID J:137469]

Recher M; Burns SO; de la Fuente MA; Volpi S; Dahlberg C; Walter JE; Moffitt K; Mathew D; Honke N; Lang PA; Patrizi L; Falet H; Keszei M; Mizui M; Csizmadia E; Candotti F; Nadeau K; Bouma G; Delmonte OM; Frugoni F; Fomin AB; Buchbinder D; Lundequist EM; Massaad MJ; Tsokos GC; Hartwig J; Manis J; Terhorst C; Geha RS; Snapper S; Lang KS; Malley R; Westerberg L; Thrasher AJ; Notarangelo LD. 2012. B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice. Blood 119(12):2819-28. [PubMed: 22302739]  [MGI Ref ID J:182528]

Sabri S; Foudi A; Boukour S; Franc B; Charrier S; Jandrot-Perrus M; Farndale RW; Jalil A; Blundell MP; Cramer EM; Louache F; Debili N; Thrasher AJ; Vainchenker W. 2006. Deficiency in the Wiskott-Aldrich protein induces premature proplatelet formation and platelet production in the bone marrow compartment. Blood 108(1):134-40. [PubMed: 16522820]  [MGI Ref ID J:135572]

Schachtner H; Calaminus SD; Sinclair A; Monypenny J; Blundell MP; Leon C; Holyoake TL; Thrasher AJ; Michie AM; Vukovic M; Gachet C; Jones GE; Thomas SG; Watson SP; Machesky LM. 2013. Megakaryocytes assemble podosomes that degrade matrix and protrude through basement membrane. Blood 121(13):2542-52. [PubMed: 23305739]  [MGI Ref ID J:195436]

Shcherbina A; Cooley J; Lutskiy MI; Benarafa C; Gilbert GE; Remold-O'Donnell E. 2010. WASP plays a novel role in regulating platelet responses dependent on alphaIIbbeta3 integrin outside-in signalling. Br J Haematol 148(3):416-27. [PubMed: 19863535]  [MGI Ref ID J:182121]

Shimizu M; Nikolov NP; Ueno K; Ohta K; Siegel RM; Yachie A; Candotti F. 2012. Development of IgA nephropathy-like glomerulonephritis associated with Wiskott-Aldrich syndrome protein deficiency. Clin Immunol 142(2):160-6. [PubMed: 22079330]  [MGI Ref ID J:180407]

Sims TN; Soos TJ; Xenias HS; Dubin-Thaler B; Hofman JM; Waite JC; Cameron TO; Thomas VK; Varma R; Wiggins CH; Sheetz MP; Littman DR; Dustin ML. 2007. Opposing effects of PKCtheta and WASp on symmetry breaking and relocation of the immunological synapse. Cell 129(4):773-85. [PubMed: 17512410]  [MGI Ref ID J:143627]

Snapper SB; Meelu P; Nguyen D; Stockton BM; Bozza P; Alt FW; Rosen FS; von Andrian UH; Klein C. 2005. WASP deficiency leads to global defects of directed leukocyte migration in vitro and in vivo. J Leukoc Biol 77(6):993-8. [PubMed: 15774550]  [MGI Ref ID J:98603]

Solomon S; Rajasekaran N; Jeisy-Walder E; Snapper SB; Illges H. 2005. A crucial role for macrophages in the pathology of K/B x N serum-induced arthritis. Eur J Immunol 35(10):3064-73. [PubMed: 16180250]  [MGI Ref ID J:113499]

Strom TS; Turner SJ; Andreansky S; Liu H; Doherty PC; Srivastava DK; Cunningham JM; Nienhuis AW. 2003. Defects in T-cell-mediated immunity to influenza virus in murine Wiskott-Aldrich syndrome are corrected by oncoretroviral vector-mediated gene transfer into repopulating hematopoietic cells. Blood 102(9):3108-16. [PubMed: 12855574]  [MGI Ref ID J:188838]

Wang C; Morley SC; Donermeyer D; Peng I; Lee WP; Devoss J; Danilenko DM; Lin Z; Zhang J; Zhou J; Allen PM; Brown EJ. 2010. Actin-bundling protein L-plastin regulates T cell activation. J Immunol 185(12):7487-97. [PubMed: 21076065]  [MGI Ref ID J:167468]

Westerberg L; Greicius G; Snapper SB; Aspenstrom P; Severinson E. 2001. Cdc42, Rac1, and the Wiskott-Aldrich syndrome protein are involved in the cytoskeletal regulation of B lymphocytes. Blood 98(4):1086-94. [PubMed: 11493455]  [MGI Ref ID J:95909]

Westerberg L; Larsson M; Hardy SJ; Fernandez C; Thrasher AJ; Severinson E. 2005. Wiskott-Aldrich syndrome protein deficiency leads to reduced B-cell adhesion, migration, and homing, and a delayed humoral immune response. Blood 105(3):1144-52. [PubMed: 15383456]  [MGI Ref ID J:104994]

Westerberg L; Wallin RP; Greicius G; Ljunggren HG; Severinson E. 2003. Efficient antigen presentation of soluble, but not particulate, antigen in the absence of Wiskott-Aldrich syndrome protein. Immunology 109(3):384-91. [PubMed: 12807484]  [MGI Ref ID J:84131]

Westerberg LS; Dahlberg C; Baptista M; Moran CJ; Detre C; Keszei M; Eston MA; Alt FW; Terhorst C; Notarangelo LD; Snapper SB. 2012. Wiskott-Aldrich syndrome protein (WASP) and N-WASP are critical for peripheral B-cell development and function. Blood 119(17):3966-74. [PubMed: 22411869]  [MGI Ref ID J:183884]

Westerberg LS; de la Fuente MA; Wermeling F; Ochs HD; Karlsson MC; Snapper SB; Notarangelo LD. 2008. WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function. Blood 112(10):4139-47. [PubMed: 18772454]  [MGI Ref ID J:142432]

Worth AJ; Metelo J; Bouma G; Moulding D; Fritzsche M; Vernay B; Charras G; Cory GO; Thrasher AJ; Burns SO. 2013. Disease-associated missense mutations in the EVH1 domain disrupt intrinsic WASp function causing dysregulated actin dynamics and impaired dendritic cell migration. Blood 121(1):72-84. [PubMed: 23160469]  [MGI Ref ID J:194094]

Zhang H; Schaff UY; Green CE; Chen H; Sarantos MR; Hu Y; Wara D; Simon SI; Lowell CA. 2006. Impaired integrin-dependent function in Wiskott-Aldrich syndrome protein-deficient murine and human neutrophils. Immunity 25(2):285-95. [PubMed: 16901726]  [MGI Ref ID J:113468]

de Noronha S; Hardy S; Sinclair J; Blundell MP; Strid J; Schulz O; Zwirner J; Jones GE; Katz DR; Kinnon C; Thrasher AJ. 2005. Impaired dendritic-cell homing in vivo in the absence of Wiskott-Aldrich syndrome protein. Blood 105(4):1590-7. [PubMed: 15494425]  [MGI Ref ID J:96605]

de la Fuente MA; Sasahara Y; Calamito M; Anton IM; Elkhal A; Gallego MD; Suresh K; Siminovitch K; Ochs HD; Anderson KC; Rosen FS; Geha RS; Ramesh N. 2007. WIP is a chaperone for Wiskott-Aldrich syndrome protein (WASP). Proc Natl Acad Sci U S A 104(3):926-31. [PubMed: 17213309]  [MGI Ref ID J:119234]

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* $3300.00
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* $4290.00
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
   002448 129S1/SvImJ (approximate)
   000691 129X1/SvJ (approximate)
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


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 Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


(6.8)