immune system
• attenuation of transition from double negative to double positive T-cells
• partial blockage between DN3 and DN4
• TCRbeta assembly issue
• problem with Vbeta to DJbeta joining
• also problems in VDdelta to Jdelta joining
|
• B cell lymphopoiesis impaired at an early stage
|
• pro B cells are normal
• pre B cells are reduced 4 fold in number
• blockade occurs when IgH assembly should be taking place
• impaired VH to DJH joining leads to a 10 fold reduction in completion of heavy chain assembly
|
neoplasm
hematopoietic system
• attenuation of transition from double negative to double positive T-cells
• partial blockage between DN3 and DN4
• TCRbeta assembly issue
• problem with Vbeta to DJbeta joining
• also problems in VDdelta to Jdelta joining
|
• B cell lymphopoiesis impaired at an early stage
|
• pro B cells are normal
• pre B cells are reduced 4 fold in number
• blockade occurs when IgH assembly should be taking place
• impaired VH to DJH joining leads to a 10 fold reduction in completion of heavy chain assembly
|
endocrine/exocrine glands
• attenuation of transition from double negative to double positive T-cells
• partial blockage between DN3 and DN4
• TCRbeta assembly issue
• problem with Vbeta to DJbeta joining
• also problems in VDdelta to Jdelta joining
|