title: Coordinately increased lysozymuria and lysosomal enzymuria induced by maleic acid.
abstract: During the acute renal tubular dysfunction of Fanconi syndrome and type 2 renal tubular acidosis (FS/RTA2) induced by maleic acid in the unanesthetized dog, we observed: 30 minutes after the onset of FS/RTA2, the urinary excretion of lysosomal enzymes, N-acetyl-beta-glucosaminidase (NAG), beta-glucuronidase (beta-gluc) and beta-galactosidase (beta-galac), increased simultaneously with the anticipated increase in renal clearance of lysozyme; the severities of all these hyperenzymurias increased rapidly, progressively, and in parallel, all reaching a peak some 60 to 80 minutes after their onset; thereafter, while the FS/RTA2 continued undiminished in severity, the severity of the hyperenzymurias decreased rapidly, greatly, progressively, and in parallel; and sodium phosphate loading strikingly attenuated the FS/RTA2 and the hyperenzymurias. Thus, the maleic acid-induced FS/RTA2 is attended by an acute reversible-complex derangement in the renal tubular processing of proteins that: affects not only lysozyme which is normally filtered, but also NAG and other lysosomal enzymes, which are not; and is to some extent functionally separable from that of FS/RTA2. The findings suggest that the derangements in renal processing of lysozyme and lysosomal enzymes are linked, and that a phosphate-dependent metabolic abnormality in the proximal tubule can participate in the pathogenesis of both these derangements and the FS/RTA2.
mesh_terms: D000118:Acetylglucosaminidase / Q000652:urine*; D000141:Acidosis, Renal Tubular / Q000139:chemically induced / Q000201:enzymology / Q000652:urine; D000818:Animals; D004285:Dogs; D005198:Fanconi Syndrome / Q000139:chemically induced / Q000201:enzymology / Q000652:urine; D005260:Female; D005696:Galactosidases / Q000652:urine*; D005966:Glucuronidase / Q000652:urine*; D006596:Hexosaminidases / Q000652:urine*; D007275:Injections, Intravenous; D007674:Kidney Diseases / Q000139:chemically induced / Q000652:urine*; D007677:Kidney Function Tests; D008298:Maleates / Q000008:administration & dosage / Q000494:pharmacology*; D008657:Metabolic Clearance Rate; D009113:Muramidase / Q000652:urine*; D010710:Phosphates / Q000008:administration & dosage / Q000378:metabolism; D001616:beta-Galactosidase / Q000652:urine*
chemical_list: D008298:Maleates; D010710:Phosphates; C030272:maleic acid; D005696:Galactosidases; D006596:Hexosaminidases; D009113:Muramidase; D001616:beta-Galactosidase; D005966:Glucuronidase; D000118:Acetylglucosaminidase; C018279:sodium phosphate