The target of the circulating ABBA autoantibodies is LDL receptor-related protein 2 (LRP2) also known as “Megalin”.
ABBA is a rare autoimmune disease associated with acute kidney injury and tubulointerstitial damage. Histologically, IgG–positive immune deposits are found in tubular basement membranes by immunofluorescence (IF). When indirect IF is done using patient’s serum and normal kidney controls, there is reaction of the autoantibodies with the proximal tubular brush borders.
Larsen CP, et al. LDL-Receptor Related Protein 2 (Megalin) as a Target Antigen in Human Kidney Anti-Brush Border Antibody Disease. J Am Soc Nephrol, 2018; 29:644-653
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