Journal
JOURNAL OF CLINICAL IMMUNOLOGY
Volume 21, Issue 5, Pages 310-327Publisher
SPRINGER/PLENUM PUBLISHERS
DOI: 10.1023/A:1012284402054
Keywords
IgA nephropathy; IgA; O-linked glycans; aberrant glycosylation; immune complexes
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Funding
- NIDDK NIH HHS [DK49358, DK57750, DK49368] Funding Source: Medline
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Several new findings emerged recently from biochemical, genetic, and molecular studies of patients with IgA nephropathy. It appears that immunoglobulin A1-secreting cells of IgA nephropathy patients produce increased amounts of aberrantly glycosylated IgA1 in which the O-linked glycans in the hinge region are deficient in the content of galactose. The galactose-deficient IgA1 in the circulation is recognized by naturally occurring antibodies with anti-glycan specificity, and immune complexes are formed. These circulating immune complexes escape hepatic degradation and eventually are deposited in the kidney mesangium. Resident mesangial cells bind the IgA-containing immune complexes with the involvement of a novel IgA receptor and become activated. A familial form of IgA nephropathy has been linked to chromosome 6q22-23. Recent progress in molecular analyses of IgA nephropathy thus defines this disease as an autoimmune process with a novel IgA mesangial receptor and certain genetically determined traits.
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