| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 8713292 | Journal of Allergy and Clinical Immunology | 2018 | 67 Pages |
Abstract
We describe a monogenic defect leading to a specific PC deficiency in human subjects, expanding our knowledge about the pathogenesis of antibody deficiencies.
Keywords
UPRCVIDCD40LIVIgPPLXBP1STransloconspliced X-box binding protein 1B cell–activating factor of the TNF familyATFC/EBP homologous proteinPKR-like ER kinaseSmall interfering RNAsiRNAinositol-requiring enzyme 1αEndoplasmic reticulum stressprotein translocationIntravenous immunoglobulinBAFFCHOPplasma cellendoplasmic reticulumactivating transcription factorCD40 ligandMultiple myelomawild-typeCalcium homeostasisUnfolded protein responsePADPERKAntibody deficiencyPrimary antibody deficiencyCommon variable immunodeficiency
Related Topics
Life Sciences
Immunology and Microbiology
Immunology
Authors
Desirée PhD, Marie-Christine MSc, Sarah MSc, Andrés PhD, Linlin MD, Michele MD, PhD, Alla PhD, Janine MSc, Johannes MD, Sandra PhD, Stephan Rusch, Manfred PhD, Alejandro A. PhD, Stefan PhD, Roger PhD, Adolfo MD, PhD, Hongzhi PhD, Fang PhD, Bodo MD,