Physiology of IgA and IgA Deficiency

@article{CunninghamRundles2004PhysiologyOI,
  title={Physiology of IgA and IgA Deficiency},
  author={Charlotte Cunningham-Rundles},
  journal={Journal of Clinical Immunology},
  year={2004},
  volume={21},
  pages={303-309}
}
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… Expand
Selective IgA Deficiency
  • L. Yel
  • Medicine, Biology
  • Journal of Clinical Immunology
  • 2009
TLDR
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. Expand
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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References

SHOWING 1-10 OF 75 REFERENCES
Immunoglobulin levels in saliva in individuals with selective IgA deficiency: Compensatory IgM secretion and its correlation with HLA and susceptibility to infections
TLDR
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. Expand
Intravascular and mucosal immunoglobulin A: two separate but related systems of immune defense?
TLDR
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. Expand
Anti-IgA antibodies in selective IgA deficiency and in primary immunodeficient patients treated with gamma-globulin.
TLDR
Sera from 106 blood donors, 40 patients with primary immunodeficiencies treated with gamma-globulin, and 46 patients with selective IgA deficiency were analyzed by an enzyme-linked immunosorbent assay for anti-IgA antibodies, suggesting that at least in some immunODeficient patients the elevated amounts of anti-G class antibodies are not related to the administration of exogenous IgA. Expand
Correlation of milk precipitins with IgA deficiency.
TLDR
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. Expand
Targeted deletion of the IgA constant region in mice leads to IgA deficiency with alterations in expression of other Ig isotypes.
TLDR
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. Expand
Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID)
TLDR
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. Expand
B‐Cell Activation in Duodenal Mucosa after Oral Cholera Vaccination in IgA Deficient Subjects with or without IgG Subclass Deficiency
TLDR
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. Expand
Inadequacy of musocal IgM antibodies in selective IgA deficiency: Excretion of attenuated polio viruses is prolonged
TLDR
It is concluded that the mucosal IgM antibodies of IgA-deficient individuals eliminate polioviruses less efficiently than do the IgA antibodies of normal individuals. Expand
Celiac sprue and immunodeficiency states: a 25-year review.
TLDR
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. Expand
Immunoglobulin A (IgA): molecular and cellular interactions involved in IgA biosynthesis and immune response.
TLDR
The impact of new information concerning IgA physiology on the immune system is discussed, which suggests that IgA should not be considered only as an isotype providing specific humoral protection of mucosal surfaces but as an integral component of the entire immune system. Expand
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