title: BAFF, APRIL and human B cell disorders.
abstract: B cells require signals from multiple sources for their development from precursor cells, and differentiation into effector cells. BAFF has been identified as a critical regulator of B cell development and differentiation. Defects in the production of BAFF and/or expression of its receptors have been associated with a diverse array of human immunopathologies characterised by perturbed B cell function and behaviour, including autoimmunity, malignancy, and immunodeficiency. This review will discuss the role of BAFF in the pathogenesis of these human immune disorders. It will also highlight relevant differences between the function of BAFF in humans and mice and the impact of this on the therapeutic utility of BAFF antagonists in the treatment of different human diseases.
authors.affiliation: Centenary Institute of Cancer Medicine and Cell Biology, Newtown, NSW, Australia. s.tangye@garvan.org.au
authors.forename: Stuart G
authors.initials: SG
authors.lastname: Tangye
authors.forename: Vanessa L
authors.initials: VL
authors.lastname: Bryant
authors.forename: Amanda K
authors.initials: AK
authors.lastname: Cuss
authors.forename: Kim L
authors.initials: KL
authors.lastname: Good
mesh_terms: D000818:Animals; D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use; D017209:Apoptosis; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism; D053264:B-Cell Activating Factor / Q000037:antagonists & inhibitors / Q000276:immunology*; D053265:B-Cell Activation Factor Receptor / Q000276:immunology; D053301:B-Cell Maturation Antigen / Q000276:immunology; D016175:B-Lymphocyte Subsets / Q000166:cytology / Q000276:immunology* / Q000473:pathology; D002470:Cell Survival; D016207:Cytokines / Q000502:physiology; D006801:Humans; D017578:Immunoglobulin Class Switching; D007153:Immunologic Deficiency Syndromes / Q000276:immunology; D007166:Immunosuppressive Agents / Q000494:pharmacology / Q000627:therapeutic use; D015448:Leukemia, B-Cell / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism; D008213:Lymphocyte Activation; D008221:Lymphoid Tissue / Q000276:immunology; D016393:Lymphoma, B-Cell / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism; D008252:Macaca fascicularis; D051379:Mice; D008805:Mice, Inbred A; D018345:Mice, Knockout; D009363:Neoplasm Proteins / Q000276:immunology; D013045:Species Specificity; D053303:Transmembrane Activator and CAML Interactor Protein / Q000276:immunology; D053300:Tumor Necrosis Factor Ligand Superfamily Member 13 / Q000276:immunology*
chemical_list: D000970:Antineoplastic Agents; D053264:B-Cell Activating Factor; D053265:B-Cell Activation Factor Receptor; D053301:B-Cell Maturation Antigen; D016207:Cytokines; D007166:Immunosuppressive Agents; D009363:Neoplasm Proteins; C108467:TNFRSF13B protein, human; C435926:TNFRSF13C protein, human; C086966:TNFRSF17 protein, human; C114631:TNFSF13 protein, human; C505719:TNFSF13B protein, human; C486361:Tnfrsf13b protein, mouse; C490176:Tnfrsf17 protein, mouse; C505720:Tnfsf13b protein, mouse; D053303:Transmembrane Activator and CAML Interactor Protein; D053300:Tumor Necrosis Factor Ligand Superfamily Member 13