JAX
Mouse Strain Datasheet - 003292
129S6/SvEvTac-Wastm1Sbs/J
Also Known As: WASP-
These Was knock-out mice exhibit reduced peripheral blood lymphocyte counts and platelet numbers along with development of chronic colitis. They are suitable for use in applications related to the study of Wiskott-Aldrich syndrome in humans.
Donating Investigator
Dr. Frederick W. Alt, Children's Hospital
Genetic overview
| Genetic Background | Generation |
|---|---|
|
|
Wastm1Sbs
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted (Null/Knockout) | Was | Wiskott-Aldrich syndrome |
Research Applications
- Hematological Research
- Immunology, Inflammation and Autoimmunity Research
- Internal/Organ Research
Detailed 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 Suggestions
Approximate Controls
Additional Information
Selected References
- 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: 9697838MGI: J:48836
Wastm1Sbs
Allele Symbol: Wastm1Sbs
| Allele Name | targeted mutation 1, Scott B Snapper |
|---|---|
| Allele Type | Targeted (Null/Knockout) |
| Allele Synonym(s) | Was-; wasp- |
| Gene Symbol and Name | Was, Wiskott-Aldrich syndrome |
| Gene Synonym(s) | IMD2; SCNX; THC; THC1; U42471; U42471; WASP; WASPA; Wasp; Wasp; Wiskott-Aldrich syndrome protein; expressed sequence U42471 |
| Strain of Origin | 129S6/SvEvTac |
| Chromosome | X |
| 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. |
| Mutations Made By | Dr. Scott Snapper, Massachusetts General Hospital |
Disease Terms
Research Areas By Genotype
This mouse can be used to support research in many areas including:
Genotype: 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
- Gastrointestinal Defects
Mammalian Phenotype Terms by Genotype
Genotype: 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
- 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)
- 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)
- 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)
- 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)
hematopoietic system phenotype
- abnormal bone marrow cell morphology/development
- 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)
- 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)
- 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)
- 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)
- in addition, mutant BMMC show reduced Ca2+ mobilization and deficient JNK activation after IgE cross-linking (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 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)
- notably, the numbers of tissue mast cells in ears of mutant mice are comparable to those of wild-type (MGI Ref ID J:86835)
- abnormal osteoclast differentiation
- 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)
- 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)
- 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)
- 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)
- abnormal osteoclast physiology
- consistent with impaired bone resorption, mutant osteoclasts tend to form small excavations on bone slices in vitro (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 increases are detected in wild-type (MGI Ref ID J:90542)
- following ovariectomy, mutants exhibit a non-significant 16.4% of bone loss relative to wild-type (38.6%) (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)
- in addition, mutant BMMC show reduced Ca2+ mobilization and deficient JNK activation after IgE cross-linking (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 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)
- notably, the numbers of tissue mast cells in ears of mutant mice are comparable to those of wild-type (MGI Ref ID J:86835)
- abnormal osteoclast differentiation
- 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)
- 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)
- 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)
- 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)
- abnormal osteoclast physiology
- consistent with impaired bone resorption, mutant osteoclasts tend to form small excavations on bone slices in vitro (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 increases are detected in wild-type (MGI Ref ID J:90542)
- following ovariectomy, mutants exhibit a non-significant 16.4% of bone loss relative to wild-type (38.6%) (MGI Ref ID J:90542)
skeleton phenotype
- abnormal osteoclast differentiation
- 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)
- 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)
- 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)
- 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)
- abnormal osteoclast physiology
- consistent with impaired bone resorption, mutant osteoclasts tend to form small excavations on bone slices in vitro (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 increases are detected in wild-type (MGI Ref ID J:90542)
- following ovariectomy, mutants exhibit a non-significant 16.4% of bone loss relative to wild-type (38.6%) (MGI Ref ID J:90542)
Genotype: 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)
- dendritic cells fail to exhibit an increase in phagocytosis of latex beads unlike 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)
- 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 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 thymocyte activation
- migratory responses to CCL19 or CXCL12 are reduced compared to in wild-type cells (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)
- unlike wild-type thymocytes, single positive (SP) thymocytes fail to proliferate after CD3 stimulation (MGI Ref ID J:125309)
- decreased NK cell number
- in the spleen (MGI Ref ID J:153247)
- decreased T cell number
- 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 anti-double stranded DNA antibody level
- at a low titer (MGI Ref ID J:153247)
- increased follicular B cell number
- (MGI Ref ID J:153247)
- increased leukocyte 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 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 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 thymocyte activation
- migratory responses to CCL19 or CXCL12 are reduced compared to in wild-type cells (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)
- unlike wild-type thymocytes, single positive (SP) thymocytes fail to proliferate after CD3 stimulation (MGI Ref ID J:125309)
- decreased NK cell number
- in the spleen (MGI Ref ID J:153247)
- decreased T cell number
- 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 leukocyte 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)
- thrombocytopenia
- (MGI Ref ID J:153247)
endocrine/exocrine gland phenotype
- abnormal thymocyte activation
- migratory responses to CCL19 or CXCL12 are reduced compared to in wild-type cells (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)
- unlike wild-type thymocytes, single positive (SP) thymocytes fail to proliferate after CD3 stimulation (MGI Ref ID J:125309)
Genotype: 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
- B cells exhibit increased IgA production after stimulation with LPS, TGF-beta1, IL-4, and IL-5 (MGI Ref ID J:180407)
- elevation in serum IgA, both in young and old 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)
- reduced ratio of sialylated and galactosylated IgA in the serum, indicating that glycosylation of IgA is abnormal in mutants (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 glomerular mesangium morphology
- mutants older than 6 months of age exhibit hump-like mesangial and paramesangial deposits (MGI Ref ID J:180407)
- abnormal kidney morphology
- mutants exhibit renal injury with increasing severity with advancing age (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)
- 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)
- mesangial cell hyperplasia
- mutants older than 6 months of age, exhibit increased mesangial cellularity with or without endocapillary proliferation (MGI Ref ID J:180407)
hematopoietic system phenotype
- increased IgA level
- B cells exhibit increased IgA production after stimulation with LPS, TGF-beta1, IL-4, and IL-5 (MGI Ref ID J:180407)
- elevation in serum IgA, both in young and old 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)
- reduced ratio of sialylated and galactosylated IgA in the serum, indicating that glycosylation of IgA is abnormal in mutants (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)
Genotype: Wastm1Sbs/Wastm1Sbs
either: (involves: 129S6/SvEvTac) or (involves: 129S6/SvEvTac * C57BL/6)
neoplasm
- neoplasm
- young mutant mice develop neither hematopoeitic malignancies nor eczema (MGI Ref ID J:48836)
digestive/alimentary phenotype
- abnormal crypts of Lieberkuhn morphology
- colitis
- crypts of Lieberkuhn abscesses
- mutants with severe colitis exhibit crypt abscesses (MGI Ref ID J:48836)
endocrine/exocrine gland phenotype
- abnormal crypts of Lieberkuhn morphology
- crypts of Lieberkuhn abscesses
- mutants with severe colitis exhibit crypt abscesses (MGI Ref ID J:48836)
hematopoietic system phenotype
- decreased B cell number
- (MGI Ref ID J:48836)
- decreased T cell number
- (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)
- 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)
- thrombocytopenia
immune system phenotype
- colitis
- decreased B cell number
- (MGI Ref ID J:48836)
- decreased T cell number
- (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)
- 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)
- 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)
Genotype: 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)
- dendritic cells fail to exhibit an increase in phagocytosis of latex beads unlike 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)
- 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
- 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 anti-double stranded DNA antibody level
- at a low titer (MGI Ref ID J:153247)
- increased follicular B cell number
- (MGI Ref ID J:153247)
- increased leukocyte 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
- 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 leukocyte 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)
- thrombocytopenia
- (MGI Ref ID J:153247)
The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain
Genotype: Wastm1Sbs/Wastm1Sbs
involves: 129S6/SvEvTac * C57BL/6
immune system phenotype
- abnormal B cell physiology
- decreased marginal zone B cell number
- 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)
- 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)
- 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 autoantibody 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
- abnormal megakaryocyte morphology
- decreased marginal zone B cell number
- 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)
- 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)
- 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)
- thrombocytopenia
- (MGI Ref ID J:182528)
Genotype: Wastm1Sbs/Wastm1Sbs
involves: C57BL/6 * CBA
immune system phenotype
- abnormal B cell physiology
- 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)
- 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)
hematopoietic system phenotype
- abnormal B cell physiology
- 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)
- 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)
Genotype: Wastm1Sbs/Wastm1Sbs
involves: BALB/c
immune system phenotype
- 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)
- 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)
- 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)
- 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)
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)
Genotype: Wastm1Sbs/Y
involves: 129S6/SvEvTac * C57BL/6
immune system phenotype
- abnormal B cell physiology
- decreased marginal zone B cell number
- 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)
- 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)
- 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 autoantibody 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
- decreased marginal zone B cell number
- 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)
- 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)
- 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)
- thrombocytopenia
- (MGI Ref ID J:182528)
References
- 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: 9697838MGI: 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: 15265894MGI: J:92036
Additional - Wastm1Sbs related
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