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      Defective Proximal Tubule Lysosomal Acidification by Bence Jones Proteins

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          Proximal tubule handling of two human Bence Jones proteins (neutral and acidic BJP) was evaluated using protein A-gold labelling. After 30 min of acute light-chain infusion into 6 rats (alone or in combination with dinitrophenyl-aminopropyl-methylamine [DAMP]), kidney biopsies were processed for immunoelectron microscopy. Antibodies directed at monoclonal lambda light chains, mannose-6-phosphate cation-independent receptor (MPR) and DAMP were used.Labelling density (number of pA-gold particles/µm<sup>2</sup>), expressed as median (25–75 percentiles), differed (p < 0.05) between the two BJP, being 94.5 (32.9–212.5) vs. 19.4 (3.7–45.6) pA-gold/µm<sup>2</sup> in endocytic vacuoles, and 297.3 (207.1–382.1) vs. 83.2 (16.6–197.0) pA-gold/µm<sup>2</sup> in non-vacuolar electrondense endosome-lysosome structures. Labelling density for MPR was 47.7 (22.2–84.6) vs. 4.0 (2.7–6.3) pA-gold/µm<sup>2</sup>. The area of MPR-labelled structures was also different, i.e.: 0.2 (0.1–0.4) vs. 0.9 (0.5–1.8) µm<sup>2</sup>. The endosome-lysosome pH distribution range differed significantly: 6.8 (6.4–7.0) vs. 6.3 (5.8–7.0). There was a significant accumulation of neutral BJP in endocytic structures, an acidification deficit of pre-lysosomes/lysosomes and MPR retention, suggestive of defective receptor recycling with this BJP. Interference with the physiological process of lysosomal acidification may be an important mechanism of higher nephrotoxicity in some BJP.

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          Méthode permettant l'étude conjuguée des propriétés électrophorétiques et immunochimiques d'un mélange de protéines. Application au sérum sanguin

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            Membrane traffic in endocytosis: insights from cell-free assays.

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              Detection of Bence-Jones protein by isoelectricfocussing of unconcentrated urine followed by nitrocellulose blotting and immunoperoxidase staining


                Author and article information

                Nephron Exp Nephrol
                Cardiorenal Medicine
                S. Karger AG
                December 1998
                06 November 1998
                : 6
                : 6
                : 514-521
                a Laboratório de Fisiopatologia Renal da Faculdade de Medicina, b Laboratório de Patologia Molecular da Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil
                20566 Exp Nephrol 1998;6:514–521
                © 1998 S. Karger AG, Basel

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                Page count
                Figures: 4, Tables: 2, References: 40, Pages: 8
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                Original Paper

                Cardiovascular Medicine, Nephrology

                Endo-lysosomal system, Multiple myeloma, Bence Jones proteins


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