期刊
JOURNAL OF CLINICAL IMMUNOLOGY
卷 28, 期 -, 页码 S56-S61出版社
SPRINGER/PLENUM PUBLISHERS
DOI: 10.1007/s10875-007-9163-2
关键词
IgA deficiency; autoimmunity clinical; evolution; Fc receptor; physiopathology
类别
Both systemic and organ-specific autoimmune diseases are major manifestations of IgA deficiency (IgAD), the most common primary immunodeficiency. In addition, to discuss the clinical findings of IgAD patients, we proposed a hypothesis to explain the high association with autoimmune phenomena. Based on observations, interactions of monomeric IgA with Fc alpha RI result in a partial phosphorylation of FcR gamma-associated FcaRI, notably in the immunoreceptor tyrosine-based activation motif (ITAM) inducing the recruitment of the SHP-1 tyrosine phosphatase. This leads to deactivation of several activating pathways of the immune system including immunoreceptors that bear ITAM motif and ITAM-independent receptors. Consequently, inflammatory reactions and auto-immune process would be prevented.
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