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Protein Kinase C δ: a Gatekeeper of Immune Homeostasis

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 mutati...

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Autores principales: Salzer, Elisabeth, Santos-Valente, Elisangela, Keller, Bärbel, Warnatz, Klaus, Boztug, Kaan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018258/
https://www.ncbi.nlm.nih.gov/pubmed/27541826
http://dx.doi.org/10.1007/s10875-016-0323-0
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author Salzer, Elisabeth
Santos-Valente, Elisangela
Keller, Bärbel
Warnatz, Klaus
Boztug, Kaan
author_facet Salzer, Elisabeth
Santos-Valente, Elisangela
Keller, Bärbel
Warnatz, Klaus
Boztug, Kaan
author_sort Salzer, Elisabeth
collection PubMed
description 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.
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spelling pubmed-50182582016-09-26 Protein Kinase C δ: a Gatekeeper of Immune Homeostasis Salzer, Elisabeth Santos-Valente, Elisangela Keller, Bärbel Warnatz, Klaus Boztug, Kaan J Clin Immunol CME Review 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. Springer US 2016-08-19 2016 /pmc/articles/PMC5018258/ /pubmed/27541826 http://dx.doi.org/10.1007/s10875-016-0323-0 Text en © The Author(s) 2016 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
spellingShingle CME Review
Salzer, Elisabeth
Santos-Valente, Elisangela
Keller, Bärbel
Warnatz, Klaus
Boztug, Kaan
Protein Kinase C δ: a Gatekeeper of Immune Homeostasis
title Protein Kinase C δ: a Gatekeeper of Immune Homeostasis
title_full Protein Kinase C δ: a Gatekeeper of Immune Homeostasis
title_fullStr Protein Kinase C δ: a Gatekeeper of Immune Homeostasis
title_full_unstemmed Protein Kinase C δ: a Gatekeeper of Immune Homeostasis
title_short Protein Kinase C δ: a Gatekeeper of Immune Homeostasis
title_sort protein kinase c δ: a gatekeeper of immune homeostasis
topic CME Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5018258/
https://www.ncbi.nlm.nih.gov/pubmed/27541826
http://dx.doi.org/10.1007/s10875-016-0323-0
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