mortality/aging
• 100% mortality by 40 weeks of age
|
growth/size/body
immune system
small thymus
(
J:36561
)
• mostly of double positive T cells
|
• fourfold decrease in CD4+CD8- single positive cells
|
reproductive system
azoospermia
(
J:36561
)
• lack of maturing sperm within the seminiferous tubules and of mature sperm in the tubule lumen, the epididymis, and ductus deferens
|
• testes were structurally abnormal
|
small testis
(
J:36561
)
|
neoplasm
• mostly of double positive T cells
|
• some develop malignant thymomas between 3 and 4 months of age
|
cellular
azoospermia
(
J:36561
)
• lack of maturing sperm within the seminiferous tubules and of mature sperm in the tubule lumen, the epididymis, and ductus deferens
|
• 3 fold increase in radiation induced chromosomal aberrations
|
• increased sensitivity of thymocytes and splenocytes to ionizing radiation
|
• fibroblasts exhibited nearly 6.5-fold more chromosomal breaks per metaphase than controls
|
nervous system
• survival of Purkinje cells in culture less than 60% of controls at 4 days and about 40% at 10 days
• survival of cultured cells improves if cultured with cerebellar astroglial cell
• develop simpler dendritic arboritization with reduced secondary branching
• nitroxide free radicals improve survival and arborization
|
endocrine/exocrine glands
small thymus
(
J:36561
)
• mostly of double positive T cells
|
• testes were structurally abnormal
|
small testis
(
J:36561
)
|
hematopoietic system
small thymus
(
J:36561
)
• mostly of double positive T cells
|
• fourfold decrease in CD4+CD8- single positive cells
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
ataxia telangiectasia | DOID:12704 |
OMIM:208900 |
J:36561 |