B cell development and primary immunodeficiencies with hypogammaglobulinemia.
The differentiation of B cells along the pathway of B cell development has been well characterized. In the bone marrow, the differentiation from pro-B cells to immature B cells can be defined by several surface antigens, such as a surrogate light chain. Immature B cells become mature B cells and then circulate in the peripheral blood as naive B cells. In the peripheral lymphoid tissues, naive B cells differentiate into memory B cells, which express the CD27 molecule, or plasma cells. Primary immunodeficiencies with hypogammaglobulinemia are caused by defects of the specific molecules which are needed for the B cell differentiation. Recent studies of the genes responsible for such immunodeficiencies have clarified B cell development as well as their pathogenesis. We discuss here the molecules affecting the B cell development and primary immunodeficiencies with hypogammaglobulinemia.
Hokibara
S
S
Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
Agematsu
K
K
Komiyama
A
A
D000361:Agammaglobulinemia / Q000150:complications*; D001402:B-Lymphocytes / Q000166:cytology / Q000276:immunology*; D002454:Cell Differentiation; D006412:Hematopoietic Stem Cells / Q000166:cytology / Q000276:immunology; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007156:Immunologic Memory