Analysis Tools
mortality/aging
|
• lifespan is shortened in conventional housing, however, in an SPF environment homozygotes can live to more than one year
|
immune system
| N |
• in contrast to homozygotes on the NOD background, spleen cell suspensions from homozygous CB17 mice exhibit NK cell activity
|
|
• spleen cells do not proliferate in response to LPS
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|
• splenic T cells do not proliferate in response to concanavalin A or to a one-way mixed lymphocyte reaction
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|
• in mice injected i.p. with cells from submandibular gland tissue of diseased Faslpr mice, inflammatory lesions are observed in salivary and lacrimal glands
• infiltrating cells are CD4+ and Vbeta8+ with a minority being CD4+ and Vbeta6+
• mice injected with cells pretreated with anti-CD4 or anti-Vbeta8 do not develop inflammatory lesions or only minor lesions are seen in parotid, submandibular, sublingual and lacrimal glands in majority of transplanted mice
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|
• the remaining thymocytes that express the Thy1 marker are larger than normal thymocytes
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• lymphocytic cortex is not evident in thymus
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small thymus
(
J:6958
)
|
• 1/10th to 1/100th normal size
|
thymus cyst
(
J:6958
)
|
• mucinous cysts are occasionally found
|
|
• homozygotes are leukopenic
|
absent B cells
(
J:6958
)
|
• B cells cannot be detected in the spleen with Ig or Lyb-8 (Cd22) markers
|
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• pre-B cells cannot be detected in the bone marrow
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• splenic follicles are devoid of lymphocytic cells
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• Peyers patches are rarely visible
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• lymph nodes have only a few lymphoid cells, however, sinuses are well-formed and contain macrophages
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• lymphid organs are 1/10th or less of normal size
• lymph organs consist mostly of supportive tissue with varying numbers of fibroblasts, macrophages and histiocytes
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• mice are unable to produce specific antibody to two T-independent antigens
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• 31/206 mice tested have low levels of serum immunoglobulin, all 31 have an IgG isotype and of these, 17 also have an IgM isotype
(J:6958)
• the remaining 175/206 mice do not have detectable serum immunoglobulin
(J:6958)
• only 1/11 homozygotescan produce greater than 1 ug/ml serum Ig at up to 100 days of age, however, 21/29 homozygotes produce greater than 1 ug/ml between 100-200 days of age
(J:22026)
|
|
• hypogammaglobulinemic
|
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• following injection of human T lymphoblastoid cells, 40% of nucleated spleen cells are of human origin by 4 weeks post injection
|
|
• homozygotes do not reject full thickness skin allografts
(J:6958)
• 1/5 homozygotes (5-6 weeks of age) reject orthotopic tail skin allografts throughout a 3 month observation period
(J:22026)
|
hematopoietic system
| N |
• in contrast to homozygotes on the NOD background, homozygous CB17 mice exhibit normal erythrocyte mean cell volumes
|
|
• spleen cells do not proliferate in response to LPS
|
|
• splenic T cells do not proliferate in response to concanavalin A or to a one-way mixed lymphocyte reaction
|
|
• the remaining thymocytes that express the Thy1 marker are larger than normal thymocytes
|
|
• lymphocytic cortex is not evident in thymus
|
small thymus
(
J:6958
)
|
• 1/10th to 1/100th normal size
|
thymus cyst
(
J:6958
)
|
• mucinous cysts are occasionally found
|
|
• homozygotes are leukopenic
|
absent B cells
(
J:6958
)
|
• B cells cannot be detected in the spleen with Ig or Lyb-8 (Cd22) markers
|
|
• pre-B cells cannot be detected in the bone marrow
|
|
• splenic follicles are devoid of lymphocytic cells
|
|
• 31/206 mice tested have low levels of serum immunoglobulin, all 31 have an IgG isotype and of these, 17 also have an IgM isotype
(J:6958)
• the remaining 175/206 mice do not have detectable serum immunoglobulin
(J:6958)
• only 1/11 homozygotescan produce greater than 1 ug/ml serum Ig at up to 100 days of age, however, 21/29 homozygotes produce greater than 1 ug/ml between 100-200 days of age
(J:22026)
|
|
• hypogammaglobulinemic
|
neoplasm
|
• spontaneous T cell lymphomas are found in greater than 10% of homozygotes at 5-9 months of age
• tumors arise in the thymus and are highly invasive and are transplantable
|
digestive/alimentary system
|
• in mice injected i.p. with cells from submandibular gland tissue of diseased Faslpr mice, inflammatory lesions are observed in salivary and lacrimal glands
• infiltrating cells are CD4+ and Vbeta8+ with a minority being CD4+ and Vbeta6+
• mice injected with cells pretreated with anti-CD4 or anti-Vbeta8 do not develop inflammatory lesions or only minor lesions are seen in parotid, submandibular, sublingual and lacrimal glands in majority of transplanted mice
|
endocrine/exocrine glands
|
• in mice injected i.p. with cells from submandibular gland tissue of diseased Faslpr mice, inflammatory lesions are observed in salivary and lacrimal glands
• infiltrating cells are CD4+ and Vbeta8+ with a minority being CD4+ and Vbeta6+
• mice injected with cells pretreated with anti-CD4 or anti-Vbeta8 do not develop inflammatory lesions or only minor lesions are seen in parotid, submandibular, sublingual and lacrimal glands in majority of transplanted mice
|
|
• the remaining thymocytes that express the Thy1 marker are larger than normal thymocytes
|
|
• lymphocytic cortex is not evident in thymus
|
small thymus
(
J:6958
)
|
• 1/10th to 1/100th normal size
|
thymus cyst
(
J:6958
)
|
• mucinous cysts are occasionally found
|
|
• spontaneous T cell lymphomas are found in greater than 10% of homozygotes at 5-9 months of age
• tumors arise in the thymus and are highly invasive and are transplantable
|
growth/size/body
thymus cyst
(
J:6958
)
|
• mucinous cysts are occasionally found
|
cellular
|
• spleen cells do not proliferate in response to LPS
|
|
• splenic T cells do not proliferate in response to concanavalin A or to a one-way mixed lymphocyte reaction
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, Nk cell-positive | DOID:0090013 |
OMIM:601457 |
J:6958 | |
