Show simple item record Kuijpers, TW Bende, RJ Baars, PA Grummels, A Derks, IA Dolman, KM Beaumont, T Tedder, TF van Noesel, CJ Eldering, E van Lier, RA
dc.coverage.spatial United States 2011-06-21T17:27:54Z 2010-01
dc.identifier 40231
dc.identifier.citation J Clin Invest, 2010, 120 (1), pp. 214 - 222
dc.description.abstract CD20 was the first B cell differentiation antigen identified, and CD20-specific mAbs are commonly used for the treatment of B cell malignancies and autoantibody-mediated autoimmune diseases. Despite this the role of CD20 in human B cell physiology has remained elusive. We describe here a juvenile patient with CD20 deficiency due to a homozygous mutation in a splice junction of the CD20 gene (also known as MS4A1) that results in "cryptic" splicing and nonfunctional mRNA species. Analysis of this patient has led us to conclude that CD20 has a central role in the generation of T cell-independent (TI) antibody responses. Key evidence to support this conclusion was provided by the observation that although antigen-independent B cells developed normally in the absence of CD20 expression, antibody formation, particularly after vaccination with TI antigens, was strongly impaired in the patient. Consistent with this, TI antipolysaccharide B cell responses were severely impeded in CD20-deficient mice. Our study therefore identifies what we believe to be a novel type of humoral immunodeficiency caused by CD20 deficiency and characterized by normal development of antigen-independent B cells, along with a reduced capacity to mount proper antibody responses.
dc.format.extent 214 - 222
dc.language ENG
dc.language.iso en_US en_US
dc.relation.ispartof J Clin Invest
dc.relation.isversionof 10.1172/JCI40231
dc.subject Animals
dc.subject Antibody Formation
dc.subject Antigens, CD20
dc.subject Antigens, CD27
dc.subject Child, Preschool
dc.subject Female
dc.subject Humans
dc.subject Immunoglobulin D
dc.subject Mice
dc.subject Mice, Inbred C57BL
dc.subject Receptors, Antigen, B-Cell
dc.subject T-Lymphocytes
dc.subject Vaccination
dc.title CD20 deficiency in humans results in impaired T cell-independent antibody responses.
dc.title.alternative en_US
dc.type Journal Article
dc.description.version Version of Record en_US 2010-1-0 en_US
duke.description.endpage 222 en_US
duke.description.issue 1 en_US
duke.description.startpage 214 en_US
duke.description.volume 120 en_US
dc.relation.journal Journal of Clinical Investigation en_US
pubs.issue 1
pubs.organisational-group /Duke
pubs.organisational-group /Duke/School of Medicine
pubs.organisational-group /Duke/School of Medicine/Basic Science Departments
pubs.organisational-group /Duke/School of Medicine/Basic Science Departments/Immunology
pubs.organisational-group /Duke/School of Medicine/Clinical Science Departments
pubs.organisational-group /Duke/School of Medicine/Clinical Science Departments/Pediatrics
pubs.organisational-group /Duke/School of Medicine/Clinical Science Departments/Pediatrics/Pediatrics, Allergy and Immunology
pubs.organisational-group /Duke/School of Medicine/Institutes and Centers
pubs.organisational-group /Duke/School of Medicine/Institutes and Centers/Duke Cancer Institute
pubs.publication-status Published
pubs.volume 120
dc.identifier.eissn 1558-8238

Files in this item

This item appears in the following Collection(s)

Show simple item record