Mouse Genome Informatics
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    Sh2d1atm1Cpt/Sh2d1atm1Cpt
B6.129S4-Sh2d1atm1Cpt
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• the number of transitional-2 B lymphocytes in the lymph nodes is decreased
• the number of CD21-Igm-B220+ and CD21-CD23-B220+ B cells is increased
• the frequency of NKT cells in the lymph node, spleen, liver and thymus is reduced
• CD1d-restricted NKT cells are absent
• IgG viral and T-dependent antigen-specific antibody responses are impaired; however, no defect in T-independent responses is seen
• in specific pathogen-free conditions basal serum levels of IgG1 are decreased
• in specific pathogen-free conditions serum IgG2a levels are increased
• after viral infection reduced primary antigen-specific IgM responses are seen
• after immunization with TNP-KLH/alum reduced serum titers of all antigen specific IgM isotypes were seen

hematopoietic system
• the number of transitional-2 B lymphocytes in the lymph nodes is decreased
• the number of CD21-Igm-B220+ and CD21-CD23-B220+ B cells is increased
• the frequency of NKT cells in the lymph node, spleen, liver and thymus is reduced
• CD1d-restricted NKT cells are absent
• in specific pathogen-free conditions basal serum levels of IgG1 are decreased
• in specific pathogen-free conditions serum IgG2a levels are increased
• after viral infection reduced primary antigen-specific IgM responses are seen
• after immunization with TNP-KLH/alum reduced serum titers of all antigen specific IgM isotypes were seen

Mouse Models of Human Disease
OMIM IDRef(s)
Lymphoproliferative Syndrome, X-Linked, 1; XLP1 308240 J:97388 , J:97690