Protein Kinase C δ: a Gatekeeper of Immune Homeostasis
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- 作者：Elisabeth Salzer ; Elisangela Santos-Valente…
- 关键词：PRKCD ; systemic lupus erythematosus ; autoimmunity ; immunodeficiency
- 刊名：Journal of Clinical Immunology
- 出版年：2016
- 出版时间：October 2016
- 年：2016
- 卷：36
- 期：7
- 页码：631-640
- 全文大小：545 KB
- 刊物类别：Biomedical and Life Sciences
- 刊物主题：Biomedicine
Immunology
Infectious Diseases
Internal Medicine
Medical Microbiology - 出版者：Springer Netherlands
- ISSN：1573-2592
- 卷排序：36
文摘
Human autoimmune disorders present in various forms and are associated with a life-long burden of high morbidity and mortality. Many different circumstances lead to the loss of immune tolerance and often the origin is suspected to be multifactorial. Recently, patients with autosomal recessive mutations in PRKCD encoding protein kinase c delta (PKCδ) have been identified, representing a monogenic prototype for one of the most prominent forms of humoral systemic autoimmune diseases, systemic lupus erythematosus (SLE). PKCδ is a signaling kinase with multiple downstream target proteins and with functions in various signaling pathways. Interestingly, mouse models have indicated a special role of the ubiquitously expressed protein in the control of B-cell tolerance revealed by the severe autoimmunity in Prkcd−/− knockout mice as the major phenotype. As such, the study of PKCδ deficiency in humans has tremendous potential in enhancing our knowledge on the mechanisms of B-cell tolerance.