Pathophysiology of IgA Nephropathy
| Autor: | Turki Al Hussain, Mohammed Akhtar, Maged H. Hussein, Hadeel Al Mana |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
biology Chemistry 030232 urology & nephrology Glomerular mesangium Glomerulonephritis Glomerulonephritis IGA medicine.disease Isotype Pathology and Forensic Medicine Proinflammatory cytokine Nephropathy Pathogenesis 03 medical and health sciences 030104 developmental biology 0302 clinical medicine Immune system Immunology medicine biology.protein Humans Anatomy Antibody |
| Zdroj: | Advances in anatomic pathology. 24(1) |
| ISSN: | 1533-4031 |
| Popis: | Immunoglobulin (Ig)A nephropathy is the most prevalent primary chronic glomerular disease in the world. Studies of molecular and cellular interactions involved in the pathogenesis of IgA nephropathy have revealed several inherent abnormalities in the production and subsequent handling of IgA1. In patients with this disease, altered glycan structures in the unique hinge region of the heavy chains of IgA1 molecules lead to the exposure of antigenic determinants, which are recognized by naturally occurring antiglycan antibodies of the IgG and/or IgA1 isotype. In addition, due to a homing abnormality there is a gradual shift of mucosal IgA1 producing lymphoplasma cells from mucosal lymphoid tissue to bone marrow resulting in excess production of mucosal-type IgA1 in the systemic circulation. As a result, nephritogenic immune complexes form in the circulation and deposit in the glomerular mesangium. Deposited immune complexes induce proliferation of resident mesangial cells with increased production of extracellular matrix proteins. A number of inflammatory cytokines produced by the mesangial cells damage the filtration barrier resulting in hematuria and proteinuria ultimately leading to progressive renal damage. |
| Databáze: | OpenAIRE |
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