Physiology of IgA and IgA Deficiency

  title={Physiology of IgA and IgA Deficiency},
  author={Charlotte Cunningham-Rundles},
  journal={Journal of Clinical Immunology},
Although secretory immunoglobulin A (IgA) is important in mucosal immunity, selective IgA deficiency is the most common primary immunodeficiency of humans. In most cases this defect is not associated with any illness. The reasons for this are unknown, but other immunological compensations might provide sufficient or complete restitution. Alternatively, it is possible that IgA deficiency alone may not predispose to disease, but additional immunological abnormalities might be present in… 
Selective IgA Deficiency
  • L. Yel
  • Medicine, Biology
    Journal of Clinical Immunology
  • 2009
Better understanding of the production and function of IgA is essential in elucidating the disease mechanism in IgA deficiency, and Alterations in transmembrane activator and calcium modulator and cyclophilin ligand interactor gene appear to act as disease-modifying mutations in both IgC deficiency and common variable immunodeficiency.
Immunodeficiency and Mucosal Immunity
The clinical implications of selective IgA deficiency
Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story
The aim of this review is to examine the association between SIgAD and atopic disease and to update readers on advances over time at this important interface between allergy andSIgAD.
Innate Mechanisms in Selective IgA Deficiency
This review focuses on some important genes, including TNFRSF13B (encoding TACI), associated with SIgAD and the involvement of epigenetics, and environmental factors, including the gut microbiota, in the development ofSIgAD.
The Epidemiology and Clinical Presentations of Atopic Diseases in Selective IgA Deficiency
The aim of the study is to summarize the connection between selective IgA deficiency and atopic diseases and hypothesized that allergic diseases are more common in patients diagnosed with this particular PID.
Autoimmunity in IgA Deficiency: Revisiting the Role of IgA as a Silent Housekeeper
A hypothesis is proposed to explain the high association with autoimmune phenomena of IgA deficiency, which leads to deactivation of several activating pathways of the immune system including immunoreceptors that bear ITAM motif and ITAM-independent receptors, and inflammatory reactions and auto-immune process would be prevented.


Immunoglobulin levels in saliva in individuals with selective IgA deficiency: Compensatory IgM secretion and its correlation with HLA and susceptibility to infections
Healthy individuals with selective IgA deficiency did not have increased concentrations of salivary IgM compared to infectious-prone patients and there was no correlation found between proneness to infections and HLA antigens or between salivARY IgM or IgG levels and Hla antigen levels in this patient material.
Intravascular and mucosal immunoglobulin A: two separate but related systems of immune defense?
Estimating the amounts of IgA produced at various sites throughout the body has shown that in humans more IgA is synthesized and secreted each day than IgG and IgM combined; about one third of this IgC is secreted directly into the vascular compartment and never reaches the mucosal surfaces.
Correlation of milk precipitins with IgA deficiency.
It appears that such precipitating antibodies are more likely to develop when antibody production is impaired in the IgA class but intact in the other immunoglobulin classes.
Targeted deletion of the IgA constant region in mice leads to IgA deficiency with alterations in expression of other Ig isotypes.
Mice with targeted deletion of the IgA switch and constant regions are completely deficient in IgA and exhibit altered expression of other Ig isotypes, notably IgM, IgG2b, Igg3, and IgE, but otherwise have normal lymphocyte development, proliferative responses, and cytokine production.
Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID)
The term `selective IgAD' should be reserved for those individuals who do not have identi®able disorders which are known to be associated with low IgA levels (see below), but in many cases a simultaneous change in the IgG subclass pattern is seen with a lack of speci®c anti-polysaccharide antibodies of the Igg2 subclass.
B‐Cell Activation in Duodenal Mucosa after Oral Cholera Vaccination in IgA Deficient Subjects with or without IgG Subclass Deficiency
The study showed that oral cholera vaccination preferentially activates intestinal IgG‐producing cells in IgAD subjects, which agreed with data recently obtained by ELISPOT in the same patients with regard to antibody‐forming cells specific for cholERA toxin.
Celiac sprue and immunodeficiency states: a 25-year review.
Although this group of patients with celiac sprue is unique in character, close follow-up coupled with conscientious compliance with a gluten-free diet, remains the mainstay of treatment for these patients.
Immunoglobulin A (IgA): molecular and cellular interactions involved in IgA biosynthesis and immune response.
The structure and function of human IgA.
  • M. Kerr
  • Medicine, Biology
    The Biochemical journal
  • 1990
It is now clear that in humans much of the IgA is secreted directly into the blood and never reaches the mucosal surfaces, so secretory and serum IgA are molecules with different biochemical and immunochemical properties produced by cells with different organ distributions.