growth/size/body
immune system
• proliferative response to surface IgM crosslinking is about 4-fold lower than in wild-type
|
• pre-B cells in culture are markedly hyperproliferative in response to IL-7
|
• antigen-dependent maturation of B cells is impaired
(J:35785)
• however, T-cell differentiation and function appear normal
(J:35785)
• defective pre-B cell development in the bone marrow
(J:114816)
|
• partial block in the pre-B1-to-pre-B2 stage of early B cell development
|
• reduced cellularity of the peripheral B cell compartment and diminished numbers of B cells that recirculate to the bone marrow from the periphery
|
• exhibit reduced numbers of pre-B2 (B220+CD43-CD25+) cells and an increased proportion of B200+CD43+ pre-B1 cells
• pre-B cells exhibit reduced pre-BCR-mediated differentiation
|
• serum IgM is generally higher
|
• show a severe deficiency in the production of secondary immunoglobulin isotypes including IgG1, IgG2a, IgG2b, IgG3, IgA, and IgE in B cells, however this is not due to a failure in isotype switching process but rather to reduced levels of transcription from normally switched immunoglobulin heavy-chain loci
|
• deficiency in production of IgG1, IgG2a, IgG2b and IgG3
|
• absent germinal center
|
• germinal centers in the lymph nodes are absent
|
hematopoietic system
• proliferative response to surface IgM crosslinking is about 4-fold lower than in wild-type
|
• pre-B cells in culture are markedly hyperproliferative in response to IL-7
|
• antigen-dependent maturation of B cells is impaired
(J:35785)
• however, T-cell differentiation and function appear normal
(J:35785)
• defective pre-B cell development in the bone marrow
(J:114816)
|
• partial block in the pre-B1-to-pre-B2 stage of early B cell development
|
• reduced cellularity of the peripheral B cell compartment and diminished numbers of B cells that recirculate to the bone marrow from the periphery
|
• exhibit reduced numbers of pre-B2 (B220+CD43-CD25+) cells and an increased proportion of B200+CD43+ pre-B1 cells
• pre-B cells exhibit reduced pre-BCR-mediated differentiation
|
• serum IgM is generally higher
|
• show a severe deficiency in the production of secondary immunoglobulin isotypes including IgG1, IgG2a, IgG2b, IgG3, IgA, and IgE in B cells, however this is not due to a failure in isotype switching process but rather to reduced levels of transcription from normally switched immunoglobulin heavy-chain loci
|
• deficiency in production of IgG1, IgG2a, IgG2b and IgG3
|
cellular
• proliferative response to surface IgM crosslinking is about 4-fold lower than in wild-type
|
• pre-B cells in culture are markedly hyperproliferative in response to IL-7
|