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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Zhou, Qing Oliveira, Joao Bosco Orange, Jordan Zhao, Yongge Fan, Xiying Uzel, Gulbu Edmonds, Pamela Jain, Ashish Wang, Hong-ying Ma, Chi A. |
| Description | Author Affiliation: Wang HY ( Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, MD 20892, USA.); |
| Abstract | Mutations in the TNF family of proteins have been associated with inherited forms of immune deficiency. Using an array-based sequencing assay, we identified an autosomal-dominant deficiency in TNF-like weak inducer of apoptosis (TWEAK; TNFSF12) in a kindred with recurrent infection and impaired antibody responses to protein and polysaccharide vaccines. This mutation occurs in the sixth exon of TWEAK and results in the amino acid substitution R145C within the conserved TNF-homology domain of the full-length protein. TWEAK mutant protein formed high molecular weight aggregates under nonreducing conditions, suggesting an increased propensity for intermolecular interactions. As a result, mutant TWEAK associated with B-cell-activating factor (BAFF) protein and down-regulated the BAFF-mediated activation of the noncanonical NF-κB pathway through inhibition of p100 processing to p52, resulting in inhibition of BAFF-dependent B-cell survival and proliferation. As BAFF mediates T-cell-independent isotype switching and B-cell survival, our data implicate TWEAK as a disease-susceptibility gene for a humoral immunodeficiency. |
| ISSN | 00278424 |
| e-ISSN | 10916490 |
| Journal | Proceedings of the National Academy of Sciences of the United States of America |
| Issue Number | 13 |
| Volume Number | 110 |
| Language | English |
| Publisher | National Academy of Sciences |
| Publisher Date | 2013-03-01 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | B-Lymphocytes Immunology Genetic Diseases, Inborn Genetic Predisposition To Disease Immunologic Deficiency Syndromes Mutation, Missense Tumor Necrosis Factors Amino Acid Substitution B-Cell Activating Factor Genetics Pathology Cell Proliferation Cell Survival Child, Preschool Down-Regulation NF-kappa B P52 Subunit Clinical Trial Research Support, N.I.H., Intramural Multidisciplinary |
| Content Type | Text |
| Resource Type | Article |
| Subject | Multidisciplinary |
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