Protein Kinase C δ: a Gatekeeper of Immune Homeostasis.
J Clin Immunol
; 36(7): 631-40, 2016 10.
Article
em En
| MEDLINE
| ID: mdl-27541826
ABSTRACT
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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Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Proteína Quinase C-delta
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Homeostase
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Imunidade
Limite:
Animals
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Humans
Idioma:
En
Revista:
J Clin Immunol
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Áustria