immune system
• thymic epithelial cell differentiation is blocked at an intermediate progenitor stage at E13.5
|
• epithelial cells in the thymus form an irregular reticular network
|
• absence of the cortical domain of the thymus
|
• absence of the medullary domain of the thymus
|
small thymus
(
J:86255
)
• adult thymus is small
|
thymus cyst
(
J:86255
)
• adult thymus is cystic
|
• fetal thymic epithelial cell differentiation is delayed by as much as 2 days, followed by arrest at a phenotype equal to that of E13.5 control thymus, however thymocyte development occurs relatively normally through E17.5, with only a slight delay in double-positive cell development, and a partial block at the TNIII (triple negative (CD3-CD4-CD8-) that are CD44-CD25+) stage at E17.5
• postnatally, thymocyte development undergoes a rapid transition to an abnormal profile, particularly at the triple-negative stage
|
• decrease in the number of double-negative cells, however a higher percentage of thymocytes are double-negative compared to controls
|
• block in double-positive cell development at the fetal and adult stages; percentage of double-positive cells is decreased by 50%
|
• 85% decrease in the number and percentage of CD3+ T cells in the spleen
|
• adults exhibit a 95-98% decrease in total thymocytes numbers compared to controls
|
• 40% decrease in CD4+ single-positive cells
|
• CD4+ single-positive cell populations have fewer cells expressing TCRbeta on their surface
|
• CD8+ single-positive cell populations have fewer cells expressing TCRbeta on their surface
|
hematopoietic system
• thymic epithelial cell differentiation is blocked at an intermediate progenitor stage at E13.5
|
• epithelial cells in the thymus form an irregular reticular network
|
• absence of the cortical domain of the thymus
|
• absence of the medullary domain of the thymus
|
small thymus
(
J:86255
)
• adult thymus is small
|
thymus cyst
(
J:86255
)
• adult thymus is cystic
|
• fetal thymic epithelial cell differentiation is delayed by as much as 2 days, followed by arrest at a phenotype equal to that of E13.5 control thymus, however thymocyte development occurs relatively normally through E17.5, with only a slight delay in double-positive cell development, and a partial block at the TNIII (triple negative (CD3-CD4-CD8-) that are CD44-CD25+) stage at E17.5
• postnatally, thymocyte development undergoes a rapid transition to an abnormal profile, particularly at the triple-negative stage
|
• 85% decrease in the number and percentage of CD3+ T cells in the spleen
|
• adults exhibit a 95-98% decrease in total thymocytes numbers compared to controls
|
• decrease in the number of double-negative cells, however a higher percentage of thymocytes are double-negative compared to controls
|
• block in double-positive cell development at the fetal and adult stages; percentage of double-positive cells is decreased by 50%
|
• 40% decrease in CD4+ single-positive cells
|
• CD4+ single-positive cell populations have fewer cells expressing TCRbeta on their surface
|
• CD8+ single-positive cell populations have fewer cells expressing TCRbeta on their surface
|
integument
N |
• normal hair development
|
endocrine/exocrine glands
• thymic epithelial cell differentiation is blocked at an intermediate progenitor stage at E13.5
|
• epithelial cells in the thymus form an irregular reticular network
|
• absence of the cortical domain of the thymus
|
• absence of the medullary domain of the thymus
|
small thymus
(
J:86255
)
• adult thymus is small
|
• adults exhibit a 95-98% decrease in total thymocytes numbers compared to controls
|
thymus cyst
(
J:86255
)
• adult thymus is cystic
|
growth/size/body
thymus cyst
(
J:86255
)
• adult thymus is cystic
|