Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept thera...
Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy
About this item
Full title
Author / Creator
Lo, Bernice , Zhang, Kejian , Lu, Wei , Zheng, Lixin , Zhang, Qian , Kanellopoulou, Chrysi , Zhang, Yu , Liu, Zhiduo , Fritz, Jill M. , Marsh, Rebecca , Husami, Ammar , Kissell, Diane , Nortman, Shannon , Chaturvedi, Vijaya , Haines, Hilary , Young, Lisa R. , Mo, Jun , Filipovich, Alexandra H. , Bleesing, Jack J. , Mustillo, Peter , Stephens, Michael , Rueda, Cesar M. , Chougnet, Claire A. , Hoebe, Kasper , McElwee, Joshua , Hughes, Jason D. , Karakoc-Aydiner, Elif , Matthews, Helen F. , Price, Susan , Su, Helen C. , Rao, V. Koneti , Lenardo, Michael J. and Jordan, Michael B.
Publisher
Washington: American Association for the Advancement of Science
Journal title
Language
English
Formats
Publication information
Publisher
Washington: American Association for the Advancement of Science
Subjects
More information
Scope and Contents
Contents
Mutations in the LRBA gene (encoding the lipopolysaccharide-responsive and beige-like anchor protein) cause a syndrome of autoimmunity, lymphoproliferation, and humoral immune deficiency. The biological role of LRBA in immunologic disease is unknown. We found that patients with LRBA deficiency manifested a dramatic and sustained improvement in resp...
Alternative Titles
Full title
Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy
Authors, Artists and Contributors
Author / Creator
Zhang, Kejian
Lu, Wei
Zheng, Lixin
Zhang, Qian
Kanellopoulou, Chrysi
Zhang, Yu
Liu, Zhiduo
Fritz, Jill M.
Marsh, Rebecca
Husami, Ammar
Kissell, Diane
Nortman, Shannon
Chaturvedi, Vijaya
Haines, Hilary
Young, Lisa R.
Mo, Jun
Filipovich, Alexandra H.
Bleesing, Jack J.
Mustillo, Peter
Stephens, Michael
Rueda, Cesar M.
Chougnet, Claire A.
Hoebe, Kasper
McElwee, Joshua
Hughes, Jason D.
Karakoc-Aydiner, Elif
Matthews, Helen F.
Price, Susan
Su, Helen C.
Rao, V. Koneti
Lenardo, Michael J.
Jordan, Michael B.
Identifiers
Primary Identifiers
Record Identifier
TN_cdi_proquest_miscellaneous_1904210451
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_proquest_miscellaneous_1904210451
Other Identifiers
ISSN
0036-8075
E-ISSN
1095-9203
DOI
10.1126/science.aaa1663
