mortality/aging
• mice typically die by 3 weeks of age
|
growth/size/body
• progressive runting
|
hematopoietic system
• impaired dendritic cell maturation and development
• absence of CD4+CD8alpha- dendritic cells
|
• lower frequency of CD11chigh dendritic cells (DCs) in 2 week old mutants
• 5.6-fold reduction in the total number of splenic DCs compared to controls
• CD4+ DC population is almost absent (31-fold reduction)
• 5.5-fold reduction of CD8alpha+ DCs
• 3.3-fold reduction of double negative dendritic cells
|
• spleens are disorganized, with complete absence of normal T and B cell areas
• distinct periarteriolar or lymphoid collections are absent although small clumps of lymphocytes are scattered throughout the spleen
|
• spleens lacked T and B cell areas
|
immune system
• impaired dendritic cell maturation and development
• absence of CD4+CD8alpha- dendritic cells
|
• lower frequency of CD11chigh dendritic cells (DCs) in 2 week old mutants
• 5.6-fold reduction in the total number of splenic DCs compared to controls
• CD4+ DC population is almost absent (31-fold reduction)
• 5.5-fold reduction of CD8alpha+ DCs
• 3.3-fold reduction of double negative dendritic cells
|
• spleens are disorganized, with complete absence of normal T and B cell areas
• distinct periarteriolar or lymphoid collections are absent although small clumps of lymphocytes are scattered throughout the spleen
|
• spleens lacked T and B cell areas
|
• LPS-treated DCs do not exhibit enhanced capacity to stimulate naive T cells
• dendritic cells fail to induce peptide antigen-specific T cell responses
|
• production of IL-12 p40 by dendritic cells in response to stimulation is impaired
|
• production of IL-6 by dendritic cells in response to stimulation is impaired
|
skeleton
osteopetrosis
(
J:85808
)
• severe
|
cellular
• impaired dendritic cell maturation and development
• absence of CD4+CD8alpha- dendritic cells
|
endocrine/exocrine glands